Lupus 2014 Abstracts 449 99

March 23, 2018 | Author: alexanderthegreat07 | Category: Systemic Lupus Erythematosus, Autophagy, Interferon, Monocyte, Interleukin 10


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Lupushttp://lup.sagepub.com/ Innate and adaptive immunity Lupus 2014 23: 449 DOI: 10.1177/0961203314527038 The online version of this article can be found at: http://lup.sagepub.com/content/23/5/449 Published by: http://www.sagepublications.com Additional services and information for Lupus can be found at: Email Alerts: http://lup.sagepub.com/cgi/alerts Subscriptions: http://lup.sagepub.com/subscriptions Reprints: http://www.sagepub.com/journalsReprints.nav Permissions: http://www.sagepub.com/journalsPermissions.nav >> Version of Record - Mar 28, 2014 What is This? Downloaded from lup.sagepub.com at Institute of Marine Biology of Crete (IMBC) on October 26, 2014 Lupus (2014) 23, 449–499 http://lup.sagepub.com ABSTRACTS 9th European Lupus Meeting, April 23–26 2014, Athens, Greece Innate and adaptive immunity A002 A001 ANALYSIS OF CIRCULATING INTERFERON LEVELS IN LUPUS PATIENTS FROM MULTIPLE WORLD POPULATIONS REVEALS LOW IFN-A IN PATIENTS FROM THE ISLAND POPULATION OF CRETE Chrabot B.S.1, Zervou M.I.2, Jolly M.3, Boumpas D.T.4,5,6, Goulielmos G.N.2 and Niewold T.B.7 1 Section of Rheumatology and Gwen Knapp Center for Lupus and Immunology Research, University of Chicago, IL, USA 2Molecular Medicine and Human Genetics Section, Medical School of Crete, Heraklion, Greece 3Division of Rheumatology and Rush Lupus Clinic, Rush University Medical Center, Chicago, IL, USA 4Institute of Molecular Biology and Biotechnology, FORTH-Hellas, Voutes, Heraklion, Greece 5Biomedical Research Foundation, Academy of Athens, Greece 6Faculty of Medicine, National and Kapodestrian University of Athens, Athens, Greece 7Division of Rheumatology, Department of Immunology, Mayo Clinic, Rochester, MN, USA ACTIVATED T CELLS ENHANCE THE INTERFERON-a PRODUCTION BY RNA-IC STIMULATED PLASMACYTOID DENDRITIC CELLS Leonard D.1, Eloranta M-L.1, Hagberg N.1, Berggren O.1, Tandre K.1, Alm G.2 and Ro¨nnblom L.1 1 Department of Medical Sciences, Rheumatology, Uppsala University, Uppsala, Sweden. 2Department of Biomedical Sciences and Veterinary Public Health, Swedish University of Agricultural Sciences, Uppsala, Sweden Introduction: A prominent interferon-a (IFNa) signature is seen in several autoimmune diseases, including systemic lupus erythematosus (SLE). Aim: We asked if T cells can promote the IFNa production by plasmacytoid dendritic cells (pDCs) stimulated with RNA containing immune complexes. Patients and Methods: Human T cells were activated by anti-CD3/ CD28 antibodies or in a mixed leukocyte reaction (MLR). T cells or supernatant from T cell cultures were co-cultured with pDCs stimulated with immune complexes containing U1 snRNP particles and SLE-IgG (RNA-IC). Cells were analyzed by flow cytometry and cytokines in co-culture supernatants were depleted or blocked by monoclonal antibodies. Culture supernatants were analyzed after 20h for IFNa and other cytokines. Results: Activated T cells or supernatants from activated T cells increased the IFNa production >20-fold. After addition of cytokines to RNA-IC stimulated pDCs, GM-CSF and IL-3 was demonstrated to increase the IFNa production. This stimulatory effect was reduced after depletion of GM-CSF in supernatants from activated T cells (81%), blocking of GM-CSF (78%) or its receptor subunits CD131 and CD116 (70-75%). Supernatant from activated T cells also increased the frequency of CD80 and CD86 expressing RNA-IC stimulated pDC from 7% to 37% and 8% to 21%, respectively. Activated SLE-T cells enhanced the IFNa production in RNA-IC stimulated pDC to the same extent as activated T cells from healthy individuals. Elevated levels of serum-GM-CSF (4.2-128 pg/ml) was found in 30% of SLE patients, all characterized by increased levels of serum-IFNa (3.0-29 U/ml) Conclusion: Activated T cells enhance the IFNa production by RNAIC stimulated pDCs via GM-CSF and IL-3 and induces maturation of the pDCs. The observation that all SLE patients with measurable serum levels of GM-CSF had elevated serum levels of IFNa indicates that activated T cells may be important for the ongoing IFNa production in this disease. Introduction: Interferon alpha (IFN-a) is a primary pathogenic factor in systemic lupus erythematosus (SLE). Levels of IFN-a may differ between SLE patients from different ancestral backgrounds, and this information could be important as therapeutics are developed that target this pathway. Aim: In this study, we compare circulating IFN-a levels in SLE patients from different world populations. Patients and Methods: 364 SLE patients were included in the study: 129 African-American, 134 European-American, 41 Hispanic-American, 14 Asian American, and 53 from Crete. All patients met the American College of Rheumatology criteria for the diagnosis of SLE. Serum IFN-a activity is measured using a sensitive reporter cell line (WISH cells) via assessment of IFN-a-induced gene transcription in cells after exposure to serum. Mann-Whitney U was used for statistical comparisons between groups. Results: Mean serum IFN-a activity levels were highest in subjects of non-European ancestry (Asian-Americans - mean 26.76, AfricanAmericans – mean 23.11, Hispanic-Americans – mean 28.71). European-Americans (mean 10.0) had statistically significantly lower IFN-a activity than all of the non-European ancestry American groups (p-values for all of these comparisons <0.001). Interestingly, the subjects from Crete had the lowest IFN levels (mean 1.3), and this was statistically significantly lower than European-Americans (p¼0.005). There was a non-significant trend toward a lower rate of antidsDNA antibody positivity in the subjects from Crete as compared to European-Americans (p¼0.14). ! The Author(s), 2014. Reprints and permissions: http://www.sagepub.co.uk/journalsPermissions.nav Downloaded from lup.sagepub.com at Institute of Marine Biology of Crete (IMBC) on October 26, 2014 10.1177/0961203314527038 9th European Lupus Meeting 450 Conclusion: European ancestry was associated with lower serum IFN-a activity, and interestingly patients from Crete had lower IFN-a than European-American patients. European-Americans represent a mix of Northern and Southern European ancestry, and it is possible that Southern European ancestry is associated with lower serum IFN-a. It is also possible that environmental or disease activity factors play a role in this finding. A003 AUTOANTIBODIES TO THE CD94/NKG2A AND CD94/NKG2C RECEPTORS IN PATIENTS WITH SLE Hagberg N.1, Theorell J.2, Hjorton K.1, Eloranta M.-L.1, Bryceson Y.T.2,3 and Ro¨nnblom L.1 1 Uppsala University, Department of Medical Sciences, Uppsala, Sweden 2 Karolinska University Hospital, Department of Medicine, Huddinge, Sweden 3University of Bergen, Institute for Clinical Sciences, Bergen, Norway Introduction: Recently, we identified a patient with systemic lupus erythematosus (SLE) that harbored autoantibodies to CD94/NKG2A. CD94/NKG2A is an inhibitory NK cell receptor that binds HLA-E. Aim: To investigate the occurrence and function of autoantibodies targeting the CD94/NKG2A, CD94/NKG2C or NKG2D receptors in patients with SLE. Method: Sera from 203 SLE patients and 90 healthy individuals were analyzed for Ig-binding to murine Ba/F3 cells transfected with CD94/ NKG2A, CD94/NKG2C or NKG2D, using flow cytometry. Identified autoantibodies were characterized for interference with HLA-E-binding, effect on degranulation in response to HLA-E-transfected K562 cells, and their capacity to induce antibody-dependent cellular cytotoxicity (ADCC). The exons encoding NKG2A (KLRC1), NKG2C (KLRC2), and CD94 (KLRD1) were sequenced. The titers of antiCD94/NKG2A and anti-CD94/NKG2C autoantibodies in longitudinally sampled sera were correlated to disease activity (SLEDAI) and severity (SLICC/ACR damage index). Results: Autoantibodies to CD94/NKG2A were found in sera from seven patients. Six of these inhibited HLA-E-binding to CD94/ NKG2A, whereas one increased this binding. Two of these patients’ sera also contained Ig that bound CD94/NKG2C, with one inhibiting, and one augmenting, HLA-E-binding to CD94/NKG2C. Anti-CD94/ NKG2A and anti-CD94/NKG2C autoantibodies abrogated the HLAE-mediated inhibition of CD94/NKG2Aþ and activation of CD94/ NKG2Cþ NK cells, respectively. Furthermore, these autoantibodies facilitated the elimination of CD94/NKG2A- and CD94/NKG2Cexpressing target cells through ADCC. No unique non-synonymous sequence variations were found in KLRC1, KLRC2, or KLRCD1. The titers of anti-CD94/NKG2A and anti-CD94/NKG2C autoantibodies were associated to disease activity. Conclusions: Autoantibodies targeting the CD94/NKG2A or the CD94/NKG2C receptor are found in a subset of SLE patients. Their titers are associated to the disease activity and a more severe SLE phenotype. Mechanistically, the autoabs can affect NK cell cytotoxicity and mediate ADCC. Consequently, anti-CD94/NKG2A and antiCD94/NKG2C autoabs may contribute to the pathogenesis of SLE and our findings highlight the possible importance of NK cells in the SLE disease process. A004 AUTOPHAGY-MEDIATED TISSUE FACTOR RELEASE IN LUPUS NEUTROPHIL EXTRACELLULAR TRAPS MAY ACTIVATE THE COAGULATION CASCADE AND CONTRIBUTE TO MICROVASCULAR INJURY Frangou E.A.1, Kambas K.2, Gergianaki I.3, Karagiorgas F.1, Karabela S.1, Bertsias G.3 and Boumpas D.T.1,3,4 1 Biomedical Research Foundation of the Academy of Athens, Athens, Greece 2Democritus University of Thrace, Alexandroupolis, Greece 3 Institute of Molecular Biology and Biotechnology, Foundation of Research and Technology-Hellas, Heraklion, Greece 4Medical School, University of Athens, Athens, Greece Introduction: Using microarrays, we have shown that active lupus patients are characterized by a neutrophil signature and differential expression of microRNAs and genes regulating autophagy. We have also shown that tissue factor (TF), the main in vivo coagulation initiator, released on Neutrophil Extracellular Traps (NETs) activates the coagulation cascade by generating thrombin, and activates platelets and endothelial cells via PAR-1 signaling. We reasoned that in lupus, autophagy mediates TF release in NETs leading to endothelial injury. Aim: To investigate the role of TF-decorated NETs in SLE. Patients and Methods: Serum and neutrophils were isolated from 12 healthy donors and 10 active lupus patients. NETs were analyzed by immunofluorescence for the co-localization of DNA with myeloperoxidase or TF. Healthy neutrophils were stimulated with lupus serum with or without autophagy inhibitors. Autophagy was assessed by immunoblotting for the conversion of LC3-I to LC3-II, and by LC3 immunofluorescence. Results: Lupus neutrophils undergo increased spontaneous NET release compared to healthy neutrophils. Moreover, healthy neutrophils treated with lupus serum exhibit increased autophagy and express increased TF levels when compared to the effects of healthy serum. The increase in TF by lupus serum is blocked by autophagy inhibitors. Furthermore, healthy neutrophils treated with lupus serum exhibit increased NET generation and TF-bearing NETs, both effects reversed by autophagy inhibition. Ongoing experiments evaluate the presence of TF-bearing NETs in the kidneys of lupus-prone mice and lupus nephritis patients, and the functional implications of TF-bearing NETs on thrombin generation and activation of platelets and endothelial cells. Conclusions: Lupus serum activates healthy neutrophils towards an autophagy-mediated TF release in NETs which may activate the coagulation cascade and contribute to microvascular injury. Reference 1 Kambas K et al. Plos One 2012. Lupus Downloaded from lup.sagepub.com at Institute of Marine Biology of Crete (IMBC) on October 26, 2014 9th European Lupus Meeting 451 A005 ELIMINATION OF GRANULOCYTIC-LIKE MYELOIDDERIVED SUPPRESSOR CELLS DUE TO EXTRACELLULAR TRAP FORMATION IN LUPUS INFLAMMATORY MILIEU Vlachou K.1, Fanouriakis A.2, Glymenaki M.1, Ioannou M.1, Mintzas K.1, Boumpas D.T.1,3 and Verginis P.1,3 1 Laboratory of Autoimmunity and Inflammation, Institute of Molecular Biology and Biotechnology, Heraklion 2Department of Rheumatology, University of Crete Medical School, Heraklion, Greece 3Laboratory of Cellular Immunology and Tolerance, Biomedical Research Foundation Academy of Athens, Athens Greece Introduction/Aim: Myeloid-derived suppressor cells (MDSCs) consist of a heterogeneous population of myeloid cells that reside in the bone marrow (BM) and give rise to macrophages, dendritic cells and granulocytes. They are characterized as CD11bhighGr-1þ and are divided into granulocytic-like (Ly6Gþ) and monocytic-like (Ly6Cþ) subsets (G-MDSCs and M-MDSCs). MDSCs regulate both innate and adaptive responses. We have recently shown that G-MDSCs contribute to the resolution of autoimmune inflammation within the central nervous system. Herein, we sought to explore their role in systemic autoimmunity. Methods: Frequency and numbers of MDSCs in bone marrow (BM) and spleens of F1 lupus mice were analyzed by flow cytometry. MDSC function was determined by T cell proliferation using CFSE. Extracellular Trap formation (ETosis) was assessed in G-MDSCs by immunofluoresence staining with DAPI and myeloperoxidase (MPO) or histone H3. Frequency of HLA-DRintCD14-CD33þCD15þ MDSCs in peripheral blood of SLE patients was determined by flow cytometry. Results: Compared to mice with encephalomyelitis, we found decreased frequency of MDSCs in the BM and spleens of F1 lupus mice with established proteinuria. The frequency of MDSCs was not significantly elevated in active SLE patients as compared to inactive patients. Sorted MDSCs from lupus mice showed an impaired ability to suppress T cell responses in vitro. Interestingly, G-MDSCs from F1 lupus mice exhibited increased spontaneous ETosis in contrast to G-MDSCs from naive C57BL/6 mice. Treatment of naive B6-derived G-MDSCs with serum from diseased F1 mice but not from healthy controls significantly induced increased ETosis. Conclusion: The lupus microenvironment promotes G-MDSCs spontaneous ETosis resulting in decreased frequency and numbers. Delineation of the molecular mechanism that promotes NETosis in G-MDSCs in the context of SLE would provide meaningful insights in the regulation of aberrant autoimmune responses. A006 ENDOTHELIN 1 IN SYSTEMIC LUPUS ERHYTEMATOSUS – NOT JUST A LOCAL PATHOGEN? Saulescu I.1, Banica L.2, Matache C.2, Opris D.1, Groseanu L.1, Borangiu A.1 and Ionescu R.1 1 UMF Carol Davila Sf. Maria Hospital, 2Cantacuzino Institute Background: Endothelin 1 (ET-1) is an important factor for vascular dysfunction, in rheumatology being mostly investigated in systemic sclerosis. In vitro data showed that alpha Interferon (IFN) can induce production of ET-1, alpha IFN being recognized as one of the corn-stone molecule in SLE. This suggests interplay between them in SLE systemic endothelial dysfunction. Objective: to evaluate mRNA ET-1 expression in a SLE cohort ands its correlation with IFN inducible genes and SLE characteristics. Material and methods: we evaluated 20 patients with SLE and 12 matched by gender and age healthy donors. All subjects gave informed consent for all procedures. A complete evaluation of SLE patients was made. mRNA levels for Endothelin-1 and IFN inducible genes IFIT1 and Mx1 in peripheral blood mononuclear cells (PBMC) were evaluated. The statistical analysis was made with SPSS programme. Results: Levels of mRNA for ET-1 and alpha IFN inducible genes were significant higher in SLE then in healthy subjects (p<0.05). Moreover, we found significant correlation between expression for ET-1 and Mx1 in SLE group (p0.037). ET -1 expression was correlated with renal involvement (p0.021). The systemic effect of ET-1 is suggested also by the correlation between ET-1 expression and fatigue (p 0.007), this might being a reflection of high serum level of ET-1 and its effect on muscle oxygen supply. Conclusions: High mRNA ET-1 expression in SLE patients was correlated with Mx-1 as part of the IFN inducible genes and also with renal involvement and fatigue. This study shows that ET-1 could represent an important pathway for ‘‘systemic’’ endothelial damage that appears in SLE. Bibliography 1 George P.M., Cunningham M.E., et al. Endothelin-1 as a mediator and potential biomarker for interferon induced pulmonary toxicity’’Pulmonary Circulation 2012; 4: 501–504. 2 Alvarez D, Briassouli P, et al. ’’A novel role of Endothelin-1 in linking Toll Like Receptor7 – mediated inflammation to fibrosis in congenital heart block’’J.Biol. Chem 2011; 286: 30444–30454. A007 FOXP3þ TREGS MODULATE DC AUTOPHAGY IN A CONTACT-DEPENDENT FASHION Alissafi T.1,2, Ioannou M.1, Hatzioannou A.2, Boon L.3, Gru¨n R. J.4, Gru¨tzkau A.4, Chavakis T.5 and Verginis P.1,2 1 Institute of Molecular Biology and Biotechnology, Foundation for Research and Technology, 71300 Heraklion, Greece 2Biomedical Research Foundation, Academy of Athens, 11527 Athens, Greece 3 Bioceros BV, 3584 CM Utrecht, Netherlands 4Institute of Infection Immunology, TWINCORE, 30625 Hannover, Germany 5Department of Clinical Pathobiochemistry, Institute for Clinical Chemistry and Laboratory Medicine and Department of Internal Medicine, University Dresden, 01307, Dresden, Germany Introduction: Foxp3þ regulatory T cells (Tregs) are crucial for the establishment of tolerance and thus, hold promise for the design of immune-based therapies. However this effort has been hampered by major caveats including the lack of understanding of their molecular and cellular targets in an un-manipulated wild-type (WT) immune repertoire. Aim: To identify novel molecular and cellular targets of Treg-mediated suppression in an intact immune system. Methods: Induction of Foxp3þ Treg-mediated tolerance in WT mice was performed utilizing a novel method for sub-immunogenic delivery of antigen with the use of implanted micro-osmotic pumps. Flow cytometry was used for determining the individual cellular subsets and cell sorting and MACS beads for their isolation. Determination of autophagic markers was performed using western blotting and immunofluorescent stainings visualized with confocal. Results: Our results pointed dendritic cell (DC) autophagy as a novel molecular target of Foxp3þ Tregs. De novo generated Foxp3þ Tregs targeted the antigen-bearing inflammatory CD11cþCD11bþGr1þ DCs (iDCs) by diminishing their accumulation in the draining lymph nodes (dLNs) and by decreasing the induction of their autophagic pathway. Furthermore, ex vivo isolated Foxp3þ Treg-exposed DCs demonstrated impaired recruitment of the autophagosome protein LC3 to antigen-containing phagosomes. Finally, we revealed that Foxp3þ Treg-mediated regulation of autophagy in CD11cþ DCs in vivo was IL-10 independent but LFA-1 and CTLA-4-dependent. Lupus Downloaded from lup.sagepub.com at Institute of Marine Biology of Crete (IMBC) on October 26, 2014 9th European Lupus Meeting 452 Conclusions: Collectively, our data decipher DC autophagy as a novel Foxp3þ Treg-mediated molecular target of immune suppression that could be exploited for potential therapeutic interventions. A008 HUMAN CATHELICIDIN IN SLE: ONE LINK BETWEEN INNATE IMMUNE SYSTEM AND VASCULAR DYSFUNCTION Saulescu I.1, Banica L.2, Matache C.2, Stavaru C.2, Opris D.1, Groseanu L.1, Borangiu A.1 and Ionescu R.1 1 UMF Carol Davila Sf. Maria Hospital, 2Cantacuzino Institute Background: Type I interferons (IFNs) are one of the innate immunity mediators that are important players in SLE pathogenesis, contributing both to autoantibody response and vascular dysfunctions. Nowadays a special attention is given to the human cathelicidin (hCAP/LL-37) as major inducer of type I IFN. LL-37 acts like an imunomodulator, displaying cytotoxic and chemotactic activities and modulating apoptosis. Objective: To evaluate mRNA expression of hCAP18 in a SLE peripheral blood mononuclear cells (PBMCs) in correlation with IFN signature genes, autoantibody response and vascular damage. Material and methods: Twenty SLE patients and 12 healthy donors (HD) were enrolled. A complete evaluation of SLE patients was made. The expression of mRNA for hCap18 and for IFN signature genes (IFIT1 and Mx1) in PBMCs was evaluated by realtime PCR. The statistical analysis was performed with SPSS. Results: Expression of mRNA for hCAP18 was higher in SLE population than in HD (p¼0,055) and strongly correlated with IFN inducible gene Mx1 (p¼0.004) and with low levels of HDL cholesterol (p¼0.031). A direct correlation was identified between hCAP18 and anti-Ro antibodies (p 0.024). We establish an arbitrary score for antinuclear antibodies: antidsDNA, anti-Sm and anti-Ro antibody and a direct correlation of that and hCAP18 mRNA level was identified (p¼0.048). In contrast, hCAP18 level was inversely correlated with SLICC damage index score calculated only for vascular damage (p¼0.038). Conclusion: hCap18 is highly expressed in SLE patients being involved in the antibody production. Up-regulation of hCAP18 mRNA in SLE PBMCs seems to be induced together with that of IFN signature genes. In contrast hCAP18 expression was inversely correlated with vascular damage. This effect might be the effect of direct action on endothelial cells (ECs), reducing their apoptosis and inducing the differentiation of mature ECs from ECs progenitors. Bibliography 1 Mocsai A. ‘‘ Diverse novel functions of neutrophils in immunity, inflammation and beyond’’. J. Exp. Med 2013; 7: 1283–1299. A009 IFNa INDUCED DIFFERENTIATION AND MATURATION OF SLE MONOCYTES ARE SUBJECTED TO AUTOPHAGIC CONTROL Gkirtzimanaki K.1, Kabrani E.1, Gergianaki I.2, Bertsias G.1, Boumpas D.1,3 and Verginis P.1,3 1 Laboratory of Autoimmunity & Inflammation, IMBB, Heraklion, Greece 2Department of Rheumatology, UoC Medical School, Heraklion, Greece 3Laboratories of Inflammation and Autoimmunity, and Cellular Immunology &Tolerance, BRFAA, Athens, Greece Introduction: IFNa, a key pathogenic cytokine in SLE, is a potent inducer of monocyte differentiation into highly activated and partially mature dendritic cells that secrete large amounts of pro-inflammatory cytokines and sustain IFNa expression in an autocrine fashion. Recent data implicate autophagy in the survival and differentiation of monocytes and presentation of self-antigens. Thus, we reasoned that autophagy may mediate the effects of IFNa on SLE monocytes. Aim: To dissect the molecular mechanism governing IFNa-induced differentiation of SLE monocytes and investigate its role in self-antigen presentation. Methods: Peripheral blood CD14þ monocytes and sera from 15 active SLE patients (females, ANAþ with lupus nephritis) and 10 age matched healthy controls were studied. Healthy monocytes were incubated with rIFNa or SLE serum and autophagy induction was assessed by immunoblotting and immunofluorescence. Ex vivo induced monocyte differentiation, maturation and mitochondrial function were analysed by flow cytometry and immunofluorescence. Chloroquine and wortmanin were used as autophagy inhibitors and B18R as IFNa soluble receptor. Results: In IFNa-treated healthy primary monocytes, kinetics of LC3I, LC3II and p62 levels and LC3 localization indicate induced activation of the autophagic machinery. Upon treatment with rIFNa or lupus serum, monocytes lose CD14 expression and upregulate HLA-DR, CD86, and CD83 levels in an autophagy dependent way, since inhibition of autophagy abolishes their differentiation and partial maturation. Interestingly, autophagy upregulation in rIFNa or SLE serum treated monocytes lead to comparable changes in mitochondrial functionality to those observed in SLE monocytes, including changes in ROS production, mitochondrial polarization and VDAC protein levels. Work in progress, addresses the contribution of the IFNa-autophagy axis in lupus autoreactivity by assessing its impact on the cytosolic release of endogenous, oxidized mitDNA and autologous T-lymphocyte reactions. Conclusion: IFNa induces monocyte differentiation and maturation in an autophagy-mediated manner, affects mitochondrial quality control, and may promote lupus autoreactivity. A010 IMPAIRED DEGRADATION AND ABERRANT PHAGOCYTOSIS OF NECROTIC CELL DEBRIS IN THE PERIPHERAL BLOOD OF PATIENTS WITH SLE Vakrakou A.G.1 and Manoussakis M.N.1,2 1 Department of Pathophysiology, School of Medicine, University of Athens, Greece 2Hellenic Pasteur Institute, Athens, Greece Introduction and Aim: Aberrant removal of necrotic debris is considered a feature with inflammatory consequences in SLE. Herein, SLE patients were investigated for the capacity of their sera to degrade secondary necrotic cell remnants (SNEC) and DNA (endonuclease DNase1 activity), as well as for uptake of SNEC by blood-borne phagocytes (SNEC-phagocytosis). Patients: For comparison, specimens from unselected RA patients and from healthy blood donors (HBD) were also studied. Results: Compared to HBD, the sera from SLE patients (but not RA) were found to exhibit significantly impaired capacity for degradation of SNEC (p¼0.008) and deficient DNase1 activity (p<0.0001) that correlated inversely with the SLEDAI activity index (r¼ -0,457, p¼0.03). Ex-vivo whole blood analyses indicated that SLE patients (but not RA) also manifested significantly increased SNEC-phagocytosis by monocytes and granulocytes (p<0.0001) that correlated with SLICC disease severity index (p¼0.01). Such aberration was largely found to reside in patients’ sera and particularly the IgG. In crossadmixture experiments, the sera of SLE patients (but not of RA) induced significant SNEC-phagocytosis by normal monocytes that correlated inversely with the DNase1 activity (p<0.0001) and the capacity for SNEC degradation (p<0.05) of these sera. In line with this, the inhibition of DNase1 in HBD sera by G-actin was found to lead to significantly diminished SNEC degradation and increased SNEC uptake by healthy phagocytes (p¼0.0009), supporting the important physiologic role of serum DNase1 in the prevention of SNEC-phagocytosis. Purified serum IgG from SLE patients manifested increased binding to SNEC and, upon addition to HBD serum, prevented the physiologic SNEC degradation and increased SNEC uptake by healthy Lupus Downloaded from lup.sagepub.com at Institute of Marine Biology of Crete (IMBC) on October 26, 2014 UK Introduction: Children with lupus have more severe disease onset and greater mortality than adults (1. Conclusion: JSLE serum reduces the phagocytic ability of healthy neutrophils.5)% and 53.2 1 Department of Women’s and Children’s health.1. Alder Hey Children’s NHS Foundation Trust. Results: Significantly increased LDG expression (%meanSEM. which correlate with dsDNA antibody concentration and scores of disease activity. Lupus Downloaded from lup. Midgley A. Institute of Translational Medicine. the immune system of SLE patients may be overly exposed to the necrotic debris and thus initiate a profound inflammatory response to tissue injury. JSLE clinical episodes % Change in phagocytosis with JSLE serum compared to corresponding control serum BILAG numerical 2004 score 1 2 3 4 5 6 7 8 9 10 24 8.97.4 8. future investigation will analyse whether JSLE serum has an effect on the ability of neutrophils to phagocytose apoptotic cells. most likely due to ‘‘shielding’’ of SNEC against endonuclease digestion. pHrodo. p¼0. Ballantine L. UK 2Department of Paediatric Rheumatology. A012 JUVENILE SYSTEMIC LUPUS ERYTHEMATOSUS SERUM REDUCES PHAGOCYTIC ACTIVITY OF HEALTHY POLYMORPHONUCLEAR CELLS Sampath S. SD¼ 12. however they are implicated in a wide range of non-infectious inflammatory conditions [1].45.W.7(16.1 and Beresford M.18.2. Ottonello L. Phagocytosis was measured using pHrodo labelled E. . 0.28.1 and Beresford M. UK Introduction: Neutrophils are critical components of the innate immune system. As previous work has demonstrated significantly increased levels of apoptotic cells in JSLE patients. A011 INCREASED EXPRESSION OF LOW DENSITY GRANULOCYTES IN JUVENILE ONSET SYSTEMIC LUPUS ERYTHEMATOSUS (JSLE) PATIENTS CORRELATES WITH DISEASE ACTIVITY Midgley A.8 10.com at Institute of Marine Biology of Crete (IMBC) on October 26. Aim: This study aimed to analyse the effect of JSLE serum on phagocytic function of PMNs derived from healthy controls.05). Liverpool.7 3 0. These correlations indicate that the increased LDG expression observed in this study may have a potential role in the pathogenesis of JSLE.46% Moderate negative correlation between reduction in phagocytosis and disease activity score (BILAG). see Table 1. A statistically significant positive correlation was observed between LDG expression and disease activity indices BILAG (correlation coefficient 0.sagepub. Standard clinical data which included disease activity markers/scores were collected for each patient. Aim: To measure the expression of LDG in JSLE patients compared to controls and any correlations with disease activity. The second most prevalent transcriptional signature in JSLE patients corresponds to neutrophil-specific genes where differential expression of these genes correlate with disease activity [2]. Spearmans rank correlation coefficient.4. r2¼0. Liverpool.9 25. Patients and Method: Neutrophils and LDG’s were isolated from JSLE (n¼8) and paediatric non inflammatory control patients (n¼10) by one-step centrifugation through polymorph prep or histopaque gradient respectively.54.05) and the biomarker of dsDNA-antibodies (correlation coefficient 0.. Spearman’s rank correlation coefficient was used to assess any correlations between disease activity markers and LDG expression. p<0.41-30.1 8.3) compared to controls (2. UK 2Alder Hey Children’s NHS Foundation Trust. Results: JSLE serum from 6 patients and total of 10 different clinical episodes were investigated.1.Coli were determined and expressed as mean SD. Percentage of PMNs phagocytosing labelled E. p<0. range) was observed in JSLE patients (11. Tissue injury in neutrophilic inflammationInflamm Res 1997. 3. PMNs incubated with JSLE serum had 11% ( 12. r¼0. 2014 . Liverpool. 28% of variation in phagocytosis could be explained by BILAG scores. p<0.Coli incubated with isolated PMNs either on ice (negative control) or at 37 C for 30 minutes with 10% control and JSLE serum.9 30.6 13 0 3 3 9 10 1 5 1 3 Mean change in phagocytosis was -11%. resulting from both cell mediated and humoral factors (4.9th European Lupus Meeting 453 phagocytes. 2 Bennett L.79.3). There was moderate negative correlation (spearmans rank correlation coefficient. coefficient of determination.45.410.05). 46(10382–91): p. et alInterferon and granulopoiesis signatures in systemic lupus erythematosus bloodJ Exp Med 2003. 197(6711–23): p. r ¼ -0.001) and SLEDAI (correlation coefficient 0. Conclusions: These data indicate that upon cell necrosis. University of Liverpool.5).8 9. Cell populations were assessed and compared using flow cytometry and morphological analysis of cytospin preparations. Methods: PMNs were isolated from blood of healthy control subjects.7(16.2.1.2 1 University of Liverpool.870.4)% with JSLE and control serum respectively. Department of Women’s and Children’s Health. quantified by flow-cytometer. Department of Paediatric Rheumatology.27. whilst patient disease activity had a trend towards moderate negative correlation with this reduction in phagocytosis.683. p¼0. Conclusion: Here we observe increased expression in an abnormal subset of neutrophils in JSLE patients. There is limited data regarding neutrophil phagocytosis within juvenile-onset SLE (JSLE). Disease activity of each of the 10 clinical episodes was measured using British Isles Lupus Assessment (BILAG) index. Phagocytic function of polymorphonuclear cells (PMNs) is defective in adult-onset systemic lupus erythematosus (SLE).1. References 1 Dallegri F. .18) between the disease activity scores and the phagocytic activity.36-5. p<0. a pH-sensitive fluorescent dye emits fluorescence at low pH levels occurring postphagocytic fusion of phagosome with the lysosome. Table 1. Investigating the morphology and characteristics of these cells will determine whether LDG represent a distinct population of lupus neutrophils with specific disruptions in neutrophil development or a more activated subset present in JSLE patients. A subset of neutrophils in the peripheral circulation of SLE patients has been described [2] and termed low density granulocytes (LDG).46%) less phagocytosis compared to control serum. Mean (SD) phagocytosis was 42.. Zanola A. To assess B helper functions. Meroni P. Patients and Methods: Blood samples from 40 clinically well-characterized SLE patients and 25 healthy donors (HD) were included. Adolescent onset of lupus results in more aggressive disease and worse outcomes: Results of a nested matched case-control study within LUMINA.1. et al. G. Lupus 2008. leading to the peripheral expansion of a subpopulation with cytotoxic activity:the CD28-T cells. Spedali Civili and University of Brescia. CCR6þIL10-producing helper T cells represent a potential therapeutic target and/or diagnostic/prognostic marker of disease activity in SLE patients.01 25. 6(5348–53.01 References 1 Strioga M.01) among CD8þT-cells was detected. Results: After 12 months of treatment.2. Sciences & Comm.03 T CD4þCD45RAþCCR7-(cell/ml) 6 9. Munoz LE. Importantly. and more recently we found that they promote B cell antibody production via IL-10.and memory T-cells. a reduction of g-IFN/IL-4(from 12.7 0. Italy. Composition of different T cell subsets in peripheral blood according to surface markers expression and intracellular cytokine production were assessed by flow cytometry. Arthritis Res Ther 2012. CHildhood-onset Disease Predicts Mortality in an Adult Cohort of patients with systemic lupus erythematosus. Italy Introduction: In addition to the classic role of vitamin D(VD)on bone metabolism. Hedberg H.000UI for the first month and 50.. but it is also a well-known B cell growth and differentiation factor produced by CD4þ helper T-cells. but are distinct from follicular helper (TFH) and Th17 cells. Milan. Aim: To study the effect of VD supplementation on the circulating levels of different T-cells subsets. IL-10 is pathogenic in systemic lupus erythematosus (SLE) where it promotes autoantibody production and nephritis.April 2008. inhibiting Th-1 and Th-17 response and enhancing Th-2 and T-regulatory function. Lupus Downloaded from lup. Immunology 2011.E. Terrier B et al. Zeni S. Clin.9th European Lupus Meeting 454 References 1 Tucker LB.2. 17: 314–322. A013 PATHOGENIC ROLE OF IL-10 PRODUCING HELPER T CELLS IN SYSTEMIC LUPUS ERYTHEMATOSUS Penatti A. 5 Janko C. Pini Institute. a multiethnic US cohort (LUMINA LVII). but not of IL-17. Impaired phagocytosis by peripheral blood granulocytes in systemic lupus erythematosus. Pini Institute.1 and Geginat J. correlated with disease activity. Ex vivo sorted CCR6þ helper T cells from blood of SLE patients produced high levels of IL-10 upon co-culture with B cells and promoted IgG production.2. many data have underlined its pleiotropic effects on different cellular types. 2014 . Conclusion: VD may promote the enhancement of peripheral induced Treg cells and the production of Th2 cytokines. Uribe AG. Rheumatology Dept. Milan.1 and Tincani A. Therefore. Andreoli L. Results: IL10-producing CCR6þ memory T cells. which demonstrate a Treg-resistant B helper capacity of IL-10-producing CCR6þ T cells in SLE patients.9 34. 8: 9–12.01 7.1. Clinical and Laboratory Characteristics and LongTerm Outcome of Pediatric Systemic Lupus Erythematosus: A Longitudinal Study. et al.3 34. but not in SLE patients.1 1 Rheumatology and Clinical Immunology. Inflammatory clearance of apoptotic remnants in systemic lupus erythematosus (SLE). Italy..1.8 0. (Epub 1969/01/01.. National Institute of Molecular Genetic.1. After repeated antigenic presentation. 4 Brandt L. the frequency of CCR6þ memory T cells and serum levels of IL-10. Italy Introduction: IL-10 is a potent anti-inflammatory cytokine that contributes to the suppressive functions of regulatory T-cells. Aim: To monitor the frequency and function of IL-10-producing B helper T cells in SLE patients and to evaluate their association with disease activity. 3 G. a significant increase in the number of peripheral induced Treg cells(CD25highCD127lowCCR7-) was observed in both groups of treatment. Abrignani S.7 16. Schorn C.com at Institute of Marine Biology of Crete (IMBC) on October 26. et al. and on cytokines production in 34 patients with Systemic Lupus Erythematosus(SLE). 3 Aimee O H. A014 THE EFFECTS OF VITAMIN D SUPPLEMENTATION ON T-CELLS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS Piantoni S. Arhritis Care Res (Hoboken) 2010 August.01 TCD8þCD28-(% CD8þ) 24. IR Cell phenotype T0 SR T12 p T0 T12 p TREG CCR7-(% TREG) 30. 2University of Pavia. The frequencies of T cell subsets were correlated with disease activity (measured by the SELENASLEDAI index). Autoimmun Rev 2008.sagepub. The Journal of Pediatrics. and 18 patients with a standard regimen(SR)(25. University of Milan. A reduction of the CD8þCD28-T-cells in SLE patients was demonstrated only in the SR group (Table 1). Italy 2INGM. In the IR group of patients evaluated for cytokines production(n¼8). 22.1. but not TFH cells were significantly expanded in the peripheral blood of SLE patients as compared to HD. little is known about the effect of VD on CD28. supporting a pathogenic role of these helper T cells. Milan. Table 1. Dall’Ara F.3. Strikingly.2 <0. T cell subsets and B cells from SLE patients and HD were co-cultured ex vivo and IgG production was measured in supernatants by ELISA. 14: R221.7 ns 26.1 to 3.000UI/monthly). 62(81152–1159.2. Rheumatology Dept. We have previously shown that human CCR6þ memory T cells produce IL-10 in secondary lymphoid organs.effectormemory-T-cells was seen. Scandinavian journal of haematology 1969. including those of the immune system.9 24. 134 (1): 17-32. Autoimmunity Unit. Patients and Methods: 16 patients were supplemented for 12 months with an intensive regimen(IR) of cholecalciferol(300. Health. Scarsi M. This hypothesis is corroborated by functional experiments. Consequently. Recent studies demonstrate that VD may have an action on T cells. p¼0. Conclusion: Our results demonstrated that accumulation of IL-10-producing CCR6þCD4þ memory T cells correlates with SLE disease activity. Grossmayer GE. 2 Linda T H. CD25þ Tregs suppressed B cell help provided by CCR6þ T cells in HD. Furthermore an increase in the total amount of circulating CD4þCD45RAþCCR7.000UI monthly). Facciotti F.9 <0.L.7 19 0. At the best of our knowledge. Fernandez M.2 1 Dept. T-cells undergo a downmodulation of CD28.2. Phenotypic analysis of peripheralT-lymphocyte and the intracytoplasmatic production of IL-4 and gIFN from peripheral blood mononuclear cells was evaluated by flowcytometry. resulting in further renal damage..NETs was removed and added to mature podocytes. Alder Hey Children’s Hospital. IL-6 exerts its functions by binding to a receptor consisting of CD130 and membrane-bound IL-6 receptor (CD126).. Amann K.1. p¼0. 2 Watson L.3. including splenomegaly.. In mice TLR7-deficiency ameliorates SLE. There was a trend towards a correlation between sIL-6R and disease activity by ECLAM (Spearman r¼-0. UK 2Department of Paediatric Rheumatology. The values where correlated with each other and disease activity score (ECLAM). 38.02). Aim: To study whether TLR8 and TLR9 act in parallel or in series in the same or different cell types in controlling TLR7-mediated lupus. Fu¨rnrohr B.sagepub. The macrophages were then either unstimulated (inactive cells) or incubated with IFN-g at a concentration of 1ng/ml (active cells)þ/.In contrast to the membrane-bound receptors... BUT SHIFTED TOWARDS TRANSSIGNALLING Skwarek M. Heschel B. p¼0.W. Conclusion: Using an in-vitro model of LN. p¼0. Methods: By analyzing TLR8-. Podocytes following culture were visualised using light microscopy. 0 (0-4. 28: 363–374. Macedo A. Tullus K. p<0. Zychlinsky A. Abed U. 2014 . W. E. G. Bonnardel J.1. However. When cultured together with active macrophages and NETs. The addition of NETs further increases this upregulation and dramatically changes the morphology of the podocytes.8-109. Laube B. 10. Department of Medicine III. Kuijpers T.or TLR9-deficiency exacerbates the disease due to increased TLR7 response1.2 and Alexopoulou L..011). Conclusions: In line with published data.1. W.[1] It is involved in the early recruitment of pro-inflammatory cells to the kidney[2]. 18: 581–588.9th European Lupus Meeting 455 A015 A016 THE IMPLICATION OF NETS ON THE UPREGULATION OF MONOCYTE CHEMOATTRACTANT PROTEIN-1 IN LUPUS NEPHRITIS Vernon A. Dying for a cause: NETosis. autoantibody production. Aix-Marseille University UM2. Fantana J. Neutrophils can undergo a process called NETosis. A. Impairment of neutrophil extracellular trap degradation is associated with lupus nephritis. while TLR8.033). and Cimaz. Brinkmann V.1. Proceedings of the National Academy of Sciences of the United States of America 2010. Vandenabeele P. Lausanne. NETs were induced from isolated neutrophils using 600nm PMA. The decrease of both membrane bound IL-6 receptor chains. Pediatric Nephrology 2013.1 1 Centre d’Immunologie de Marseille-Luminy. A.1 (18.. CD126 and CD130 were closely correlated (Pearson r¼0.. and thus from CRP producti References 1 Watson L.NETs.1. both TLR8 and TLR9 control TLR7 function. serum sIL-6R was increased in SLE as compared to HC (45. possibly shifting IL-6 effects from hepatocytes (carrying CD126). Liverpool.. C. Midgley A.3)(median (range)) vs. Aim: To investigate the IL-6/IL-6R system in SLE.0001) and CD130 (4919% vs. Lippens S. Human monocytes were differentiated into macrophages using media containing monocyte colony stimulating factor..1 (20..6)(median (range)) vs.2 1 Institute of Translational Medicine (Child Health). 5 Hakkim A.. but this seems not to hold true in SLE. Vanden Berghe T.79.. Thus. TLR7. Cell Death Differ 2011. Germany Introduction: Monocyte chemoattractant protein-1 (MCP-1) has been identified as a biomarker of lupus nephritis (LN). After 48 hours the supernatant from the macrophages þ/. Results: Serum IL-6 levels of SLE patients were higher than those of HC (3 (0. mechanisms behind an antimicrobial cell death modality. Herrmann M. 107: 9813–9818.003)...1. R. D. Urinary monocyte chemoattractant protein 1 and alpha 1 acid glycoprotein as biomarkers of renal disease activity in juvenile-onset systemic lupus erythematosus.and double TLR8/9-deficient mice and primary cells we evaluated SLE development and immune responses..com at Institute of Marine Biology of Crete (IMBC) on October 26. Serum levels of IL-6 and soluble IL-6 receptor (sIL-6R) were measured by ELISA. co-culture of human podocyte cells and active macrophages resulted in significant up-regulation of MCP-1 production. Marks S. 608%. Midgley A.373). Division of Rheumatology. Beresford M.. Henri S. Pilkington C. 2 University Hospital CHUV. IL-6 is upregulated in SLE. Peripheral blood mononuclear cells (PBMC) were immediately prepared from peripheral venous blood and stained with PE-labelled anti-CD126 or anti-CD130 or control antibodies.. Jones C.B. Patients and Methods: Lymphocytes of 50 SLE patients were compared to those of 68 healthy individuals (HC). 5910%. p<0. TLR8 and TLR9 sense microbial or endogenous nucleic acids and are implicated in the development of SLE.8) pg/ml. together with the increase in soluble IL-6 receptors suggests that trans-signalling via sIL-6R may dominate in SLE..5-69. Roussel-Queval A. Lupus 2012. and Aringer M. France. A. 21: 496–501. up-regulated in the active disease.1-57) ng/ ml. 4 Remijsen Q. University of Liverpool. or soluble IL6 receptor (sIL-6R). Holt R. Results: We found that double TLR8/9-deficient (TLR8/9-/-) mice on the C57BL/6 background showed increased abnormalities characteristic of SLE. (2012) Monogenic forms of systemic lupus erythematosus: new insights into SLE pathogenesis. the podocytes produced the highest concentration of MCP-1 (4716420pg/mL. Beresford M. p<0. INSERM U1104. MCP-1 concentration (meanSEM) was measured using ELISA.1. Introduction: Systemic lupus erythematosus (SLE) is a complex autoimmune disease with diverse clinical presentations characterized by the presence of autoantibodies to nuclear components. Lower percentages of lymphocytes of SLE patients than of HC expressed CD126 (4917 (meanSD) vs. L. Urine biomarkers in juvenile-onset SLE nephritis.1 and Beresford M. Introduction: Interleukin-6 (IL-6) is closely linked to CRP. there was significantly increased MCP-1 from the podocytes was produced following the addition of active macrophages (3874292pg/mL. W. Impairment of NET degradation is associated with LN...056). Demaria O. Methods: Human podocyte cell-line with human-monocyte derived macrophages was used to create an in vitro model of (LN). Results: There was no difference in the concentration of MCP-1 from podocytes alone (1968101pg/mL) compared to in the presence of inactive macrophages (1860135pg/mL. which is found on hepatocytes and leukocytes.0001). p¼0.. frequencies of marginal zone and B1 B cells and renal disease compared to Lupus Downloaded from lup... A017 TLR8 ON DENDRITIC CELLS AND TLR9 ON B CELLS RESTRAIN TLR7-MEDIATED SPOTANEOUS AUTOIMMUNITY IN C57BL/6 MICE Desnues B.. 13288 Marseille.1.035) or NETs (4279181pg/mL: p¼0. CNRS UMR 7280. p¼0. UK THE INTERLEUKIN-6/INTRELEUKIN-6-RECEPTOR SYSTEM IS UPREGULATED IN SLE. Voll R. Wirawan E. University Clinical Center Carl Gustav Carus at the Technical University of Dresden. Switzerland. which results in the release of neutrophil extracellular traps (NETs)[4]. TLR9. p¼0. 3 Belot. Pediatric Rheumatology.[5] Aim: To use an in vitro model of LN to assess the effect of NETs on the production of MCP-1 by podocytes.0001).. Yancopoulos GD. IL-23 AND TGF-b IN SERBIAN PATIENTS WITH ATIPHOSPHOLIPID SYNDROME Popovic-Kuzmanovic D. IL-23 and TGF-b and gene polymorphisms as susceptibility markers for Primary Antiphospholipid Syndrome (PAPS) in Serbian population. 2 Popovic-Kuzmanovic D. University of Belgrade. and we have showed that IFN-a production by the plasmacytoid dendritic cell (pDC) was enhanced by crosstalk with natural killer (NK) cells and B cells. TLR8 on dendritic cells and TLR9 on B cells restrain TLR7-mediated spontaneous autoimmunity in C57BL/6 mice. Ann Rheum Dis 2008. Merrill J. Double heterozygosity polymorphisms for platelet glycoproteins Ia/IIa and IIb/IIIa increases arterial thrombosis and arteriosclerosis in patients with the antiphospholipid syndrome or with systemic lupus erythematosus..1. Serbia 2 Institute of Biology. Trajkovic V.sagepub. Demaria O. Zogovic N. J Clin Invest 2010. Serbia Aim: To investigate the serum concentrations of cytokines IL-17. Pagni PP. Increased activity of interleukin-23/interleukin-17 cytokine axis in primary antiphospholipid syndrome. Roussel-Queval A.. Aksentijevich I. University of Belgrade. B cells from TLR9-/.B cells not. Aksentijevich I. while TLR8-/.001) depending on the combination of stimulated cell types.4.. 67: 835–840. displayed as elevated serum levels of IFN-a and increased expression of type I IFN inducible genes (an IFN signature). Results: We found associations between IFN-a production and 28-107 SNPs (p<0.T.and TLR8/9-/. Alexsson A. Murphy AJ. Alexopoulou L. Immunobiology 2013 Feb.C. A018 COPYRIGHT PROTECTED. Belgrade. Traub S. Uppsala. On the cellular level. rs11209026 and rs1800471 variants in patients with PAPS compared to healthy subjects. Macedo AM. Belgrade. we found that the SLE-risk variant of the 5 bp IRF5 CGGGG-indel was associated with a lower IFN-a production. Valenzuela DM.cells responded normally..2. Rheumatology and Science for Life Laboratory. Alexopoulou L. IL-23 and TGF-b were significantly higher in PAPS patients than in the control group (p < 0. Lupus Downloaded from lup. Bonnardel J. Tovilovic G. Henri S. Conclusion: Our results reveal that TLR8 and TLR9 have an additive effect on controlling TLR7 function and TLR7-mediated lupus. 6: 458–462. TLR8 deficiency leads to autoimmunity in mice. they act on different cell types2.4 and Trajkovic V. and stimulated with RNA-containing immune complexes (IC). The SNP rs2275913 (IL17A). herpes simplex virus (HSV) or the oligonucleotide ODN2216. National Institute of Arthritis. IFN-a production was measured in the cell cultures by an immunoassay.dendritic cells were hyperesponsive to TLR7 ligand R848. 2 Desnues B. Methods: pDC. Musculoskeletal. A020 GENE POLYMORPHISM OF CYTOKINES IL-17. Belgrade. References 1 Jimenez S. rs763780 (IL17F).05. while TLR9 restrains TLR7 response on B cells.1. Zogovic N. No statistically significant differences were observed in the distribution of genotypes and alleles of the rs2275913. Sweden 2Uppsala University. Moreover.9th European Lupus Meeting 456 single TLR8-/.1. Serum concentrations of IL-17. Ro¨nnblom L.. Lately. Data from future association studies may be added to the meta-analyses to obtain more precise estimates of effect sizes. NOT FOR PUBLICATION Genetics and -omics (genomics. School of Medicine. Tovilovic G. rs763780. Curr Rheumatol Rep 2004. Cervera R. Proc Natl Acad Sci U S A 2014. while TLR9-/.. only three of these associated SNPs were shared between the cell type combinations. metagenomics.mice. NIH. 3 Horita T. Genetics of antiphospholipid syndrome. Several of the SNPs have not been associated with type I IFN production previously. Belgrade. TLR8-/. 218(2186–91.5 1 ’’Bezhanijska Kosa’’ University Medical Center.3. de Gassart A. USA 4Institute of Biological Research.1. All blood donors were genotyped with 200K ImmunoChip and a 5bp CGGGG length polymorphism in the IFN regulatory factor 5 gene (IRF5) by PCR. Espinosa G. MD. Novakovic I.. B and NK cells were isolated from peripheral blood obtained from healthy individuals. Tandre K. Tassies D. while some loci have been described earlier for their genetic association with SLE.and TLR8/9-/. TLR8 controls TLR7 function on dendritic cells. However. Stojanovich L.mice were hyperesponsive to R848. Dept. however. Plaza J.1. rs11209026 (IL-23) and rs1800471 (TGFb) was genotyped using commercial pre-synthesized TaqMan allelic discrimination assay.1 1 Uppsala University.com at Institute of Marine Biology of Crete (IMBC) on October 26. Stojanovich L. Controlling the expression and production of these cytokine may be a new approach to the treatment of PAPS and gene polymorphisms can influence the risk of illness.. 120: 3651–62.1 and Eloranta ML. Serbia 3Genetics and Genomics Branch. References 1 Demaria O. IL-23 and TGF-b were measured by commercial ELISA kits. Uppsala. Syva¨nen AC. Mann-Whitney test). Reverter J. 2014 . 111: 1497–502. Flavell RA. Dept. Novakovic I. Garcia-Criado A. Conclusion: We found a large number of genetic variants affecting the ICmediated type I IFN production that highlight the intricate regulation of the type I IFN system and can reveal new therapeutic targets for SLE.. Results and Conclusions: The levels of IL-17. Aim: We investigated whether single nucleotide polymorphisms (SNPs) associated with SLE and other autoimmune diseases have an impact on the IFN-a production by pDC. and Skin Diseases. Bethesda. Serbia 5Institute of Microbiology and Immunology. School of Medicine. of Medical Sciences. metabolomics) A019 EFFECT OF SINGLE-NUCLEOTIDE POLYMORPHISMS ON TYPE I INTERFERON PRODUCTION BY PLASMACYTOID DENDRITIC CELLS STIMULATED WITH SLE-ASSOCIATED IMMUNE COMPLEXES Berggren O. proteomics. Material and Methods: We analyzed samples of fifty patients with Primary Antiphospholipid Syndrome (PAPS) and fifty healthy controls.or TLR9-/.2. studies have shown an association between the susceptibility to SLE and several gene variants within the type I IFN system. Sweden Introduction: Systemic lupus erythematosus (SLE) and many other systemic autoimmune diseases have a persistently activated type I interferon (IFN) system. Monteagudo J. Branzk N.. of Medical Sciences. Furthermore. Molecular Medicine and Science for Life Laboratory. Aristotle University of Thessaloniki. ncont¼2452) ncont¼748) ncont¼11516) ncont¼2016) Number P<1x10-6 7 of regions PFDR<0.3.3. We analyzed gene ontology for each set of miRNA targets as well as their participation in major biological pathways. Papagianni E. Results identified differential expression of a list of miRNAs between healthy and patient samples. NC. the SLE ImmunoChip Consortium. (ncase¼557. TRANS-ANCESTRAL IMMUNOCHIP STUDY TO UNRAVEL GENETIC RISK IN SLE Cunninghame Graham D. and provides insights regarding genetic and epigenetic effects.Ther 13: 101–103. Conclusion: Trans-ancestral mapping of known autoimmune loci has revealed novel SLE-associated loci and signalling pathways important in immunological function.Res. BRFAA and Medical School. Results: Numerous regions met Immunochip-wide significance (P<1x10-6) and PFDR<0. Iliopoulos D.4.5x10-5 3. Greece 2Department of Nephrology.2. We have performed miRNA analysis in the peripheral blood and kidneys of patients with lupus nephritis (LN). though with discrete role in each tissue. miRNA target genes identified in LN participate in networks associated with renal failure. (ncase¼1872. Combination of this analysis with miRNA regulation in blood may lead to identification of novel lupus biomarkers. MAPK). al (2010) Nucleic. Top upregulated miRNA is miR-422a.02 2x10-4 References 1 Brown and Cortes (2011) Arthritis.05 (Table 1).9th European Lupus Meeting 457 A021 A022 MICRO-RNA NETWORK ANALYSIS IN LUPUS NEPHRITIS IDENTIFIES SEVERAL BIOLOGIC PROCESSES INVOLVED IN ITS PATHOGENESIS Banos A. 4Genentech Inc.3. Methods: LN biopsies and peripheral blood mononuclear cells were analyzed for expression level of human set of miRNAs. 2 Kim et. Krasoudaki E. TGF-b signaling. the RNA Polymerase II Associated Protein RPAP2. London. In each ancestry functional pathways were investigated using GSA-SNP2.1.1. which further extend our understanding of the pathogenesis of lupus.3 1 Laboratory of Autoimmunity & Inflammation. Lupus Downloaded from lup. Results: In LN.1. lupus pathogenesis. Table 1.2. Conclusion: Various miRNAs and their targets are key players in gene networks that contribute to LN. miR-21 is a common genetic regulator both in renal biopsies and the peripheral blood. cytokine signaling and transcription. respectively. National and Kapodestrian University of Athens.1 1 King’s College.1. Aim: To identify miRNA-mediated gene expression and gene networks involved in LN.01 1.001 Preliminary pathway analysis also revealed an enrichment of risk loci implicated in natural killer cell cytoxicity and in cell adhesion (Pcorrected<0. Aim: To identify novel susceptibility loci for SLE and discover molecular processes for disease we undertook a trans-ancestral association analyses and pathway analysis on the Immunochip1 in four different ethnicities. Bertsias G. CTF1.2 and Vyse T. Validation of these findings and identification of molecular biomarkers of LN and lupus molecular signature are in progress. multiple kinases (ERK. Another miR. UCLA.05 27 6 22 35 35* 13 14 PFDR<0.com at Institute of Marine Biology of Crete (IMBC) on October 26. 2014 .1.sagepub. Thessaloniki. with a specifically designed array. gene networks and disease signatures.Res 38:W90-95. Top novel associations include the transcription factor PHTF1. IMBB.1.4x10-5 2x10-3 0. Kelly J. several miRNAs presented with altered expression in renal biopsies.T.Acids. Greece 3Laboratory of Inflammation and Autoimmunity. the inner mitochondrial translocase TIMMDC1 and the secreted cytokine. using GeneCoDis and Ingenuity Pathway Analysis. Gaffney P. Table 2. 2Oklahoma Medical Research Foundation. We also compared miRNA levels between renal biopsies and the peripheral blood. (ncase¼6748. Trans-racial non-parametric meta-analysis was computed using METAL. False discovery rate (FDR) adjusted p-values were computed to account for the actual number of tests. Association was modelled using a logistic regression adjusting for admixture proportions. The strong genetic contribution to disease includes risk loci shared with other autoimmune disorders. Graham R. Stagakis I.01 0. In total 12147 cases and 16732 controls were genotyped and passed quality control (Table 1). Pcorrected for KEGG Pathway Ethnicity AA AsA EA HA NK cytotoxicity Cell adhesion 4. Cristofides A. Greece 4Division of Digestive Diseases. Frangou E.01 in all four ethnicities) (Table 2). Langefeld C. the receptor tyrosine kinase EFNA1/TNFAIP4. OA 3 Wake Forest University. We sum up the gene targets of these miRNAs through Targetscan algorithm and proceed with gene network analysis. with prevalence varying by ethnicity. USA Introduction: Gene expression represents the intermediate phenotype between DNA and disease phenotypic variation. Ethnicity AA AsA EA HA (ncase¼2970. Athens. CA Introduction: Systemic lupus erythematosus (SLE) is the prototypic autoimmune disease.4 and Boumpas D.3.7x10-6 0. the histone methyltransferase ASH1L (ASH1-Like Protein). Heraklion. MicroRNAs (miRNAs) regulate the expression of genes involved in immune activation and response of the kidney to the immune injury. Patients and Methods: SLE cases met the American College of Rheumatology criteria. which regulates KLK4 gene. Predicted gene-targets identified by bioinformatics were validated by transfection studies (luciferase assay.1. Bucharest.6. miR-422a levels were increased and KLK4 mRNA levels were reduced in the kidneys of NZB/NZW LN mice. BMC medicine [Internet]. especially those with high ANA titers.13:792–9. Micro-RNA expression was analyzed by TaqMan Low Density Arrays v1. Mihalcea S. We have previously performed micro-RNA analysis in the peripheral blood to reveal molecular pathways contributing to T-cell hyperactivity in lupus. UCLA. compared to 200 healthy controls. 2004 Jan. To our knowledge this is the first study. Kiss E. Conclusion: KLK4. Green A. Furtunescu F. overexpression of miR-422a suppressed KLK4 mRNA by 82%. Zaboulaki A.3. Except from ANA and Immunoglobulin M Rheumatoid factor.349(16):1526–33. Leszczyn´ski P. Scofield RH. Greece 2Division of Digestive Diseases. may be downregulated by miR422a in LN. First degree relatives (FDR) to SLE patients have a higher prevalence of autoantibodies and are more prone to suffer from autoimmune and rheumatic diseases (2. Dennis GJ. These results were confirmed at protein level.. Bucharest. Rubertone M V. Poland 7National Institute of Rheumatology and Physiology. and immunohistochemistry showed reduced KLK4 expression in the glomerular podocytes and tubular epithelial cells in human and murine LN. At follow-up the proportion of ANA positive FDRs had increased. Greece 5University of Athens. University Of Crete. Of the 13 ANA positive at titer 1:160 at baseline. identify associated variables. One participant developed SLE during follow-up. Rojas-Villarraga A. whereas KLK4 was increased in cells transfected with miR-422a inhibitor. Results: 193 out of 226 (85%) FDRs were alive and 143 (74%) participated. In HEK-293T cells. Conclusion: The study underscores that FDRs to SLE patients have an increased ANA prevalence compared to healthy controls and the prevalence increases during 12 years follow-up. Bertsias G. and Voss A. 2 Ca´rdenas-Rolda´n J. James J a. Junker P. Thessaloniki. These data reiterate the involvement of local factors within the kidney in determining the disease phenotype in lupus nephritis. Heegaard NHH. Patients and Methods: RNA was purified from kidney biopsies of LN patients (n¼7 proliferative. Hungary Introduction: Assessment of organ damage has become the standard outcome measure for morbidity and mortality in patients with systemic lupus erythematosus (SLE). n¼5 membranous) and unaffected tissue from renal carcinoma patients (n¼5).Registries and cohorts . et al. Deceased participants were older and with a male preponderance but no difference in antinuclear antibody (ANA) status was observed. Iliopoulos D. In 2013 a re-examination was performed. and assess socioeconomic status of patients with SLE.1.1. Budapest. Odense University Hospital. Majdan M. 2003 Oct 16. ASSESSMENT OF DAMAGE ACCRUAL AND SOCIOECONOMIC STATUS IN A LARGE INTERNATIONAL SLE COHORT Pamfil C. Laustrup H. A025 A024 A STUDY OF AUTOANTIBODIES AND SELF-REPORTED HEALTH DURING 12 YEARS FOLLOW-UP IN RELATIVES TO SLE PATIENTS Langkilde H.2. How do autoimmune diseases cluster in families? A systematic review and metaanalysis. USA 3Department of Nephrology. Perna A. The results show. Lillevang ST. References 1 Arbuckle MR. Pedersen L.7 and Rednic S.9th European Lupus Meeting 458 A023 TRANSCRIPTOMIC ANALYSIS REVEALS DECREASED EXPRESSION OF KALLIKREIN-4 IN LUPUS NEPHRITIS DUE TO UPREGULATION OF MICRO-RNA-422A Krasoudaki E. FORTH. The New England journal of medicine [Internet]. Autoantibodies may evolve years before SLE diagnosis (1). Lupus [Internet].1. Results: Nine micro-RNAs were upregulated and 15 micro-RNAs were downregulated in LN compared to control renal tissue. Lupus Downloaded from lup. McClain MT.Environmental exposures Patients and methods: In 2001 226 FDRs to a population-based lupus cohort were examined for prevalence of autoantibodies and selfreported health (4). Participants and non-participants were comparable in all baseline characteristics. Antonsen S. At baseline. Poland 6Medical University of Lublin. Denmark Introduction: Systemic Lupus Erythematosus (SLE) is an autoimmune disease characterized by presence of autoantibodies and characteristic multiorgan involvement. if previous autoantibodies and self-reported health complaints can predict autoantibody status and self-reported health complaints at follow-up. Autoantibodies and self-reported health complaints in relatives of systemic lupus erythematosus patients: a community based approach. UK 4GSK.2 and Boumpas D. 9 (69%) were ANA positive at titer 1:160 and 1 (8%) at titer 1:80 at follow-up. Romania 5Poznan Medical University.2.1 1 Iuliu Hatieganu University.T.3. that autoantibody positivity is a permanent phenomenon in certain relatives to SLE patients.5. Sørensen HT. Institute of Molecular Biology-Biotechnology. London. Cluj-Napoca. real-time PCR). Anaya J-M. Greece 4Department of Histology. Department of Rheumatology. Voss A. 2013 Apr [cited 2013 Sep 24].11. 2013 Jan [cited 2013 Aug 9]. 4 Laustrup H. In line with the human data.1. a secreted serine esterase with angiogenic and extracellular matrix remodeling properties. BioMed Central Ltd. Aim: To analyze the micro-RNA transcriptome in lupus nephritis (LN) and identify novel genes implicated in its pathogenesis.72(4):525–9. Romania 2Carol Davila University. Greece Introduction: Lupus etiopathogenesis involves aberrancies in gene expression and function in immune effector cells and in target organs.5 1 Laboratory of Autoimmunity and Inflammation. Alexopoulos E.1. Results were confirmed in the NZB/NZW murine model of LN and protein expression was assessed by immunoblotting and immunohistochemistry. where antibody statuses of FDRs to SLE patients are followed over time.4. Aristotle University of Thessaloniki. Parental history of lupus and rheumatoid arthritis and risk in offspring in a nationwide cohort study: does sex matter? Annals of the rheumatic diseases [Internet]. Romania 3GSK. Epidemiology and outcomes . significantly more FDRs were ANA positive in all titres.3.3). Nine reported to have Rheumatoid Arthritis and six reported to have other connective tissue diseases during follow-up. Development of autoantibodies before the clinical onset of systemic lupus erythematosus.0. Stagakis E. Drakos E. no antibody or self-reported health complain at baseline could convincingly predict antibody status or self-reported health complaints at follow-up.4. miR-422a showed the highest upregulation (17-fold) and was found to target kallikrein-4 (KLK4) in luciferase assay. Aim: To study. Loupasakis K. who participated in the follow-up study. 2014 .sagepub. Papagianni A. Aim: Determine the extent of damage accrual. 3 Somers EC.com at Institute of Marine Biology of Crete (IMBC) on October 26. 2. deceased Introduction: Renal involvement is a major cause of morbidity and mortality in patients with systemic lupus erythematosus.001).T.7 years (28.5. The frequency. Conclusion: Damage accrual was associated with disease activity and duration. 2014 . Compared to patients with SDI¼0 (n¼384).9th European Lupus Meeting 459 Patients and methods: The SESAME study was a prospective.001).4. 2 Rheumatology.9. observational study. clinical and histological features were recorded. Gergianaki I. tertiary referral centers are prone to referral biases. Poland¼6.1.8.2%) cases of lupus nephritis.1years and a mean disease duration of 7. 25 patients had proteinuria >300mg /24 hours. Demographic. Stratigis S. Clinical Immunology and Allergy.1. Results: Mean  standard deviation age at SLE diagnosis and disease duration were 33. University of Crete. Patients and Methods: We reviewed retrospectively the histological diagnosis of all renal biopsies performed on residents of the island of Crete (population approximately 0. Histological findings compared with gender are shown in the figure below. higher proportion of financial support and social assistance (p<0. General Hospital of Chania. but there were no available data of the weigh or height of 21 patients.1.9. et al.0  7. Greece. Clinical and laboratorial data were collected using the hospital information records.1. 12 patients had a blood pressure superior to 130/80 mmHg. 5Nephrology Department. Stratakis S.9 g/dl. by controlling disease activity and minimising reliance on corticosteroids.2 and Dafnis E.004).018).000. p¼0. and to identify factors affecting renal survival. Portugal Introduction: The pathogenesis of cardiovascular risk in SystemicErythematosus Lupus (SLE) is the result of a complex interaction between traditional risk factors. FORTH.5 mg/day. Methods: A convenient sample of outpatients with SLE attending our Rheumatology Clinic was recruited.sagepub. we identified 63 (8. Maragaki E.3. Evaggelismos Hospital. The highest frequencies within the items of SDI were recorded for patients with glucocorticoid-related/potentially-related damage.1.Aveiro. doctors should control every risk as possible. Statistical analysis was performed using Excel. Greece. Concerning cholesterol levels in this SLE patient’s cohort: 21 had LDH>100 mg/dl. corresponding to a prevalence of 17 per 100.com at Institute of Marine Biology of Crete (IMBC) on October 26. Parasiris G. Greece. 9University of Athens. Medical School. whereas in the remaining 59% kidney biopsy was performed during the course of the disease. Greece. Ntaountaki E. 32. Stylianou K. Poulidaki R. Meirinhos T. Hungary¼2) and followed-up for 12 months. Sidiropoulos E.6. In 41% of the cases. Boumpas D. Nakopoulou L. for patients and socioeconomic reasons.1. Consecutive SLE patients (N¼737) were enrolled at 19 specialized lupus centers (Romania¼11. severity and outcome of lupus nephritis differs according to patient demographic characteristics such as race. Aguiar T. 12 total Lupus Downloaded from lup. in a SLE cohort.1. this corresponds to 14% lupus patients having biopsy-proven nephritis.001).7. lower proportion of employment (p<0. J Rheumatol. disability and a more vulnerable socioeconomic status. respectively.P.5. studies in major academic. Greece. and age and between populations from different countries. Athens. Reference 1 Gladman DD. with a mean age of 41. The damage accrual group had a significantly lower proportion of subjects with anti-Ro antibodies (p¼0. Tzanakis G.6 million) during the years 1991-2013.4 years. Xydakis D.004). Venizeleio General Hospital.6. These initial SESAME findings highlight the need to reduce damage accrual. 3Institute of Molecular Biology-Biotechnology..1 1 Nephrology Department. disease specific factors and chronic inflammation. Heraklion. To minimize de cardiovascular risk in these patients. Athens. Greece. Greece. glucocorticoid dose. p<0. Perakis K. Results: From a total of 768 biopsies. General Hospital of Rethymnon. Results: 60 patients were enrolled (54 women and 6 men). 7Pathology Department. Disease damage and activity were assessed by SLICC Damage Index (SDI) and SELENA-SLEDAI.E. Cases of lupus nephritis were identified from both the Conclusions: Biopsy-proven lupus nephritis may be less common than previously reported by major academic centers. Average activity index was 5.7.3.1. Papadogiannakis A. A026 BIOPSY-PROVEN LUPUS NEPHRITIS IN A SOUTHERN EUROPEAN GENETICALLY HOMOGENOUS POPULATION Tzanakakis M.4. Since the prevalence of SLE in Crete is 123 per 100. Papadaki A. Aim: To determine the profile of lupus nephritis in a genetically homogenous population with shared environmental exposures living in both rural and urban settings.4 and chronicity index was 1. Objective:In order to implement afollow-up protocol in our SLE patients. Mean Creactive protein was 0. gender.9  12. Athens. Vardaki E.3. University of Crete. higher proportion of disability (p<0. higher proportion of medical retirement (p¼0.9 years and 8. Sotsiou F.9 years in men. or not.30:1955-9. Ambro´sio C. 13 patients had smoking habits and 22 patients were overweight.001) and a higher median dose of glucocorticoids than patients with SDI¼0 (10 vs 7. Here we report data gathered at patient inclusion.81 mg/dl and albumin 3.000 in the general adult population in Crete. 4Nephrology Department. 8Pathology Department.73 mg/dl.2.3:1.045).001).6. university Hospital as well as from smaller nephrology units in secondary care hospitals. Gakiopoulou X. Only 5 patients were insulin resistant. the authors made a transversal assessment on cardiovascular risk factors.1. Importantly. and Barcelos A. Bertsias G. Greece. Heraklion. Heraklion.7 years. and how they are controlled.0 years in women) with female:male ratio of 5. A027 CARDIOVASCULAR RISK ASSESSMENT IN A SLE COHORT – WHERE DO WE STAND? Canic¸o J. Further analyses are underway to determine long-term renal and patient outcomes in association with sociodemographic characteristics and the type and responses of administered therapies. patients with SDI1 (n¼353) had longer disease duration (p<0. deforming/erosive arthritis and scaring chronic alopecia. University of Athens. Perysinaki G. RheumatologyDepartment – Centro Hospitalar do Baixo Vouga E. inner city. Greece.. Mean creatinine was 0. 6Nephrology Department. The mean age at the time of lupus nephritis diagnosis was 31. to avoid an increase in the mortality and morbidity. nephritis was the presenting manifestation of lupus.2. 2003.. Fanouriakis A. Patients with SDI1 also had higher disease activity (p<0.none was diabetic. and a similar proportion of subjects receiving antimalarial therapy. Petra X. and were more likely to require inpatient hospitalization (39% vs 54%. Curr Rheumatol Rep 2007. and acoustic evoked potentials. Perricone C. ß2GPI (IgG/IgM) and LA. Serbia 2Institutut of Rheumatology Belgrade University. Harle´ J. 31(5756–760.2.3%. health portals(55%).7%. Marisavljevic D. Diagnosis of neurological manifestations was established by clinical findings. Miranda F. 2 Roldan JF. physical activity. 4 Stojanovich L. migraine in 29. The mean dose of corticosteroid was 5. Damjanov N. autoimmune diseases. Amital H. Antiphospholipid antibodies (aPL) analysis included analysis of aCL (IgG/IgM). aCL IgM and epilepsy (p¼0. visual. Transitient ischemic attack in 28.1. References 1 Arnson Y. contraception. psychological impact. department of internal medicine. Lupus Downloaded from lup. A focused cross-sectional online survey was also proposed to members of the community. and Valesini G. Lupus Clinic. . including Systemic Lupus Erythematosus (SLE). psychiatric features and a variety of other cerebral syndromes.-R.2%. The 10 most popular topics (836 posts) were ‘‘Lupus and [. transient global amnesia in 2. Psychiatric manifestations in systemic lupus erythematosus.4% acute ischemic encephalopathy in 7.02). within online communities dedicated to specific diseases.9th European Lupus Meeting 460 cholesterol>200 mg/dl. and 51. sun exposure.6 mg pd. Patients and methods: We analyzed 142 SLE patients with secondary APS–aAPS (90. Marisavljevic D. Neurologic manifestations of the antiphospholipid syndrome. type. long-term effects. Aim: Describe characteristics of the Carenity SLE community and identify needs and sources of information of SLE patients.9%. forums(46%). Conclusions: The Serbian National APS Registry allowed us to ascertain a significantly increased incidence for the certain neurological manifestations in SLE patients with sAPS. Autoimmun Rev 2007. multiinfarct dementia in 13. Conclusion: The results reinforce the importance of a protocolled followup that couldavoid the lack of some important clinical and laboratorial data and a better/more efficient control of co-morbidities in SLE patients. daily life. toxics. outcomes. 112 patients fulfilled the online survey. fibromyalgia.008). to prevent damage is a major goal in the management of SLE patients. weight. 7 HDL< 40 mg/dl and 5 had triglycerides>150 mg/dl. Conclusion: This study provides relevant data on the needs and sources of information of SLE patients which could be used to improve content and form to provide more relevant information. 45. 40: 97–108. A028 CEREBRAL EVENTS IN SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS WITH ANTIPHOSPHOLIPID ANTIBODIES: RESULTS FROM THE SERBIAN NATIONAL COHORT STUDY Stojanovich L.. Conti F. Zadman-Goddard G. patients’ associations’ websites(46%). Belgrade. Chekroun M.R. work. disease. lupus websites (51/77/40%). issued by the Ministry of Science of the Republic of Serbia. fatigue.. We revealed positive relationship between aCL IgG and incidence of acute ischemic encephalopathy (p¼0. Paris. All tipes of aFL are specific to detect SLE patients at risk for the cerebral events. From April to May 2013. Smiljanic D. Clin Exp Rheumatol 2013. paraclinical investigations. Serbia 3Hospital Center Zemun. chorea in 7. Belgrade. duration/cessation. Franc Introduction: There is a lack of data about needs and sources of information of patients with rare diseases. In addition to neurologic and psychiatric examination. Carenity is the first French online platform allowing peer-to-peer exchanges.9% sAPS patients.9 years) geographically distributed homogeneously throughout territory. Djokovic A. 6: 421–26. There was no positive correlation between others aPL and neurological manifestations.. Serbia Introduction: Central nervous system involvement is one of the most prominent clinical manifestations in patients with systemic lupus erythemathosus (SLE) as well as in antiphospholipid syndrome (APS) and includes thrombotic events. Sources of information were various with relative variations according to timing respectively ‘‘just before’’. Results: Overall neurological and psychiatric manifestations were present in 77. epilepsy in 18. Objectives: Demonstration of the cerebral events in patients with SLE from the Serbian National APS Registry. Rheumatology. the CNS involvement was assessed with digital electroencephalography.1. anterior spinal artery syndrome in 0. France 2Carenity. all the posts were analyzed. treatments. Massaro L. patient associations.]’’:nutrition. Rome Introduction: Published data suggest a significantly higher mortality in patients affected by Systemic Lupus Erythematosus (SLE) developing chronic damage. They reported lack of information about: 1)At diagnosis: outcome. 3 Stojanovich L. Patients and Methods: One year after creation of the SLE community. A030 CHRONIC DAMAGE IN SLE PATIENTS: RESULTS FROM A LARGE MONOCENTRIC COHORT Ceccarelli F. 2232 could be classified into specific topics. .1% were LA positive.3 1 Internal medicine.1. Therefore.0% patients had b2GPI IgM positive. .. somatosensory. Sikanich N.4% patients had aCL-IgM positive. Department of Neurology.04) and ß2GPI IgG and stroke (p¼0. Seminars in Arthritis and Rheumatism 2010. Pavlovich S.0%.1 and Smiljanich-Miljkovich D. 2)At treatment initiation: side effects. Acknowledgements: This work was supported by research grant number 175041 for 2011-2014. ‘‘just after diagnosis’’ and ‘‘before treatment initiation’’: healthcare providers (51/61/ 67%). Aix-Marseille University.7%. Sapienza Unversity.2% male) average age 47. 57. electromyoneurography. In this SLE cohort. and MRI. Djokovic A.8%.1. symptoms. there were 8 thrombotic vascular events but 5 of these patients had also antiphospholipid syndrome. ’’Bezhanijska Kosa’’ University Medical Center. patient associations (11/23/9%). Alessandri C. psychosocial aspects. hemiballismus in 0.2 and Chiche L. 40. Spinelli F. diagnosis. 12 patients were medicated with statins and 28 with angiotensin –converting enzyme inhibitors or angiotensin receptor blockers. Leccese I. 9: 109–15. From a total of 6702 posts. Results: The SLE community included 521 people (female:93%. Internet forums are understudied resources to know more about living with such diseases. The Antiphospholipid Syndrome as a Neurological Disease.. vaccines.1 1 La Conception hospital.sagepub. . 2014 . Stamenkovic B. entourage. mechanism of action.8% female and 9. Alon E.7 patients had no lipid profile in the last year.6%. symptoms and sexuality. stroke in 28. A029 CHARACTERISTICS.]’’: treatment. global management.7% patients had b2GPI IgG positive..8 years. Kontic M. Marseilles. NEEDS AND SOURCES OF INFORMATION OF A FRENCH ONLINE COMMUNITY OF SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS Meunier B. Truglia S. Association between non-thrombotic neurological and cardiac manifestations in patients with antiphospholipid syndrome. mean age:38. Brey RL.214.7% cerebellar ataxia in 0. pregnancy.9%.com at Institute of Marine Biology of Crete (IMBC) on October 26. Facebook(21%). journals/magazines (7/12/6%). Shoenfeld Y.9% sAPS patients had aCL-IgG positive. Additional topics of interest (with at least 10 posts each) were ‘‘Lupus and [. French patients blogs(31%). Internet was used to find information about SLE via search engines(74%). web forums/blogs (34/53/19%). 61... renal and neuropsychiatric involvement associated positively with SDI1. Kong KO.01). Patients with damage were significantly older. Patients with SDI1 have different clinical manifestations. while the Systemic Lupus Erythematosus Quality of Life (SLEQoL) questionnaire is disease-specific for adults with SLE. Sousa S. disease duration.4%) domains were the most commonly affected. 32. discoid rash and positivity of anti-phospholipids were predictors of damage.01.A. Cerqueira M.3 and Santos M. Roma˜o V.4 1 Special Hospital for Rheumatic Diseases Novi Sad.6. whereas the Role limitations caused by Physical problems (RP) were perceived as the worst.2. Finally. Silva C. The use of antimalarials (ever or current) showed a negative association with damage. A cross sectional analysis was made upon records of the last visit.741. range 05). The best quality of life was shown in the domains of Vitality (VT) and Bodily Pain (BP). The correlation between the SF-36 PCS score and the SLEQoL score was r¼-0. Bernardes M. The logistic regression analysis revealed the association between the neuropsychiatric damage and the presence of antiphospholipid syndrome (P¼0. A031 DAMAGE PREDICTORS IN PATIENTS FROM THE PORTUGUESE LUPUS REGISTRY Gonc¸alves M. Instituto Medicina Molecular. average age of 43. Patients and Methods: We enrolled patients affected by SLE according with ACR 1997 criteria. Health-related quality of life (HRQoL) was assessed by applying the SF-36 and SLEQoL. Silva J.648 (p<0.02). disease duration (P<0. Results: 1099 patients were studied (439 with SDI 1).3. References 1 Leong KP. Borges J.2 and Stojanovic R. and the Chi-square test and Pearson0 s correlation coefficient were used. Faro 9Hospital de Aveiro.9.70.J.26.9.com at Institute of Marine Biology of Crete (IMBC) on October 26. The SLICC/ACR damage index (SDI) measures cumulative damage and is associated with a higher morbidity and mortality rate.01). Conclusions: In this large cohort study.5. Ineˆs L. Descriptive statistics. Predictor factors for damage. serositis.sagepub.4 years. Gender and SLEDAI score at last visit were not associated with damage. A032 HEALTH-RELATED QUALITY OF LIFE IN SERBIAN LUPUS FEMALE PATIENTS USING SF-36 AND SLEQOL Stojkovic S. Terroso G. Thong BY. Conclusions: In our large SLE population we identified a mild chronic damage in 36% of evaluated patients. Lisboa 2Hospital Garcia de Orta. and with the use of glucocorticoids (P¼0.02).6%). mean SDI score was 0. many patients develop irreversible organ consequences during the course of disease. References 1 Gladman D. Aveiro.666 (p<0. Conclusions: Both SF-36 and SLEQoL were useful instruments in assessing HRQoL in Serbian lupus female patients. In multivariate analysis.2).1. In general.5.C.6.3.1. defined as SDI 1. in a large monocentric Italian cohort of SLE patients and we aimed at evaluating its associations with demographic factors. It is prudent to use both tests in order to minimize their limitations. with development of deforming/erosive arthritis in 21 subjects.58 (range 22-63). clinical features.2 months): 125 patients (35. Disease activity was assessed by the SLEDAI2K and flare was defined as an increase of SLEDAI-2K4 compared with previous visit. Portugal Introduction: Systemic lupus erythematosus (SLE) survival rate has improved dramatically. mean ageSD 42. Within SLEQoL.2. Patients and Methods: SLE patients from the Portuguese register Reuma.2. Aim: Characterization of damage and identification of damage predictors. Duarte C. The musculoskeletal was the most frequently involved system (41/ 125 patients. Lupus Downloaded from lup. 2014 . Novi Sad.001).001). mean disease durationSD 164.J.8. Coimbra 5Instituto Portugueˆs Reumatologia. Raposo A. Musculoskeletal (24.8%). antiphospholipid syndrome and Sjo¨gren’s syndrome were also more prevalent in this group. serositis. Lisboa 4 Hospitais da Universidade de Coimbra. age. Barcelos A. renal involvement. the best quality of life was noted in the domain of Treatment. Miranda L. J Rheumatol. Canas da Silva J. 1992. Almada 3 Rheumatology Research Unit.1. older age and longer disease duration.3211.9105. 44(101267–76.9th European Lupus Meeting 461 Aim: We assessed chronic damage by means of the SLICC Damage Index (SDI).7. the SLEQoL score had a better correlation with the SF-36 domains than the SF36 and SLEQoL domains had with each other. The correlation of comparable domains ranged from strong to weak correlation. discoid rash.pt/LES and available SDI were included. The association with the number of flares confirms the need to control disease activity in order to prevent the damage. clinical and demographic characteristics were found to be associated with damage. number of flares (P¼0.7. Serbia 2 General Hospital Loznica.4. the Mental Component Summary (MCS) score was better than the Physical Component Summary (PCS) score. Results: We enrolled 349 SLE patients (M/F 25/324.5.P. The correlation between the SF-36 MCS score and the SLEQoL score was r¼-0. Our nationwide study provides crucial information on damage in a homogenous South European SLE population. Chronic damage was retrospectively determined by SDI at the last visit in all the patients with at least 6 months of follow-up. Pereira da Silva J. Canha˜o H.4. Patients and Methods: The cross-sectional study included 50 female SLE patients.01.9) and between the presence of cardiovascular damage and positive test for anti.0%) and ocular (16. Serbia 3Institute of Rheumatology Belgrade 4University of Belgrade School of Medicine. Serbia Introduction: The Medical Outcomes Study Short Form-36 (SF-36) is the most common generic instrument used to measure quality of life in Systemic Lupus Erythematosus (SLE).712. had longer disease duration and later disease onset. the possible role of antiphopholipid antibodies (aPL) in the development of cardiovascular and neurological damage may suggest a tight-control in aPL positive patients. while the SLEQoL Treatment wasn’t comparable to any of the SF-36 domains. OR¼3.4. Costa L. Black ethnicity. Loznica. Results: Within SF-36. treated at the Institute of Rheumatology in Belgrade.A. Rheumatology (Oxford) 2005.b2GPI antibodies (P¼0. were determined by a multivariate logistical regression model.8%) showed a SDI1 (meanSD 1. Ponte de Lima 8Hospital de Faro.6. Hypertension. Vrbavac M. neuropsychiatric (23.1. Development and preliminary validation of a systemic lupus erythematosus-specific quality-of-life instrument (SLEQOL).3 1 Hospital de Santa Maria. et al. Belgrade. whereas Self-image and Activity were perceived as the worst. The presence of chronic damage was associated with significantly higher age (P<0. OR 6.1. Sequeira G. Porto 7Hospital Conde de Bertiandos.3.S. Lisboa 6Hospital de Sa˜o Joa˜o. Aim: To investigate the association of SF-36 and SLEQoL measurements in SLE patients. disease activity and laboratory findings (1). However. 2%.003) and serositis (0. high CACS (>400) were associated with age. Results: There were 226 (92. Demographic and clinical characteristics were obtained prospectively from study subjects.002). Patients and Methods: In a population-based predominantly Caucasian cohort we recruited 84 SLE patients and 52 healthy controls.5%..3. Taipei.1 and Voss A.6%) women and 18 (17. 2014 .7% v.D.s. Measurement of CACS was expressed in Agaston units.1. diabetes in 5. there was only limited data about the prevelance of HBV infection in SLE patients. Results from a single center. seven developed acute liver failure. All patients fulfilled the revised ACR criteria for diagnosis of SLE. as well as SLE damage. but also with SLICC. Taiwan Introduction: SLE patients are more suspesible to infection. However. Laustrup H. no one developed acute liver failure. 15-20%). 24: 1061–1065.07. National Taiwan University Hospital. Aim: The aim of this study was to evaluate the prevalence and the extent of coronary artery calcium among Danish patients with SLE compared with healthy control subjects.4% cases cause of death was SLE related or drug withdrawal.3 1 Department of Rheumatology. Diederichsen A.2.2% pts. the risk of acute liver failure in SLE patients with HBV infection was much higher than those without HBV infection.0  11. mean age 57. However. Conclusion: In a population-based SLE cohort high extent of CACS occur more frequent as compared with healthy controls. Improved survival over 24 years. hypertension in 28.05). The prevalence of HBV infection in SLE patients was 8. Clinical and laboratory indicators of disease activity were recorded at each clinic visit and entered into a database. Immunology and Rheumatology.024) as well as cigarette smoking (p¼0. Odense University Hospital. Hag I.1%. There was significantly increased risk of aucte liver failure in the HBV infection group (P < 0.4% pts had non-erosive arthritis. 93.002). It is a concern for HBV reactivation in SLE patients. Prophylactic therapy for HBV was recommended before immunosuppresant therapy. Sultan SM. SLE patients had significantly higher odds of having CS > 400 (OR¼17. 48. III. the prevalence of HBV infection in SLE patients was lower than the general polpulation in Taiwan (8. The controls were 67% women. obesities in 20. Mortality studies in systemic lupus erythematosus. and Stanisavljevic N.7% (24/279). Results: A total 369 SLE patients were evaluated. serositis in 32. 33.5 years. A035 MORTALITY PREDICTORS IN SERBIAN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS: RESULTS FROM A SINGLE CENTER Stojanovich L. 95% CI: 2.7% photosensitivity. Smoking was present in 29. dyslipidemia in 23.8%. Conclusion: Ih this retrospective study. HBV infection was defined as positive HBs-Ag. HBs-Ag had been tested in 279 patients. Am J Epidemiol 1997 Mar 1. Abu-Shakra M. 66% hematologic disorder. For SLE patients. Mortality risk factors were age 50 years (p¼0. et al. Total of 239 pts (98%) was treated with steroids.2% had CAPS. The liver prognosis of HBV infection in SLE patients is still not clear. This study provides evidence that there is increased HBV reactivation and with poor liver outcome in SLE patients. Nephritis was diagnosed in 25% of the cases. duration of disease and SLICC were assessed. hypertension and abdominal obesity. Denmark 2 Department of Cardiology. Mortality rate through 20-year period was 11. There were 5 patients died due to acute liver failure. However.25. ’’Bezhanijska Kosa’’ University Medical Center. mean age 50.76. Outcome of a cohort of 300 patients with systemic lupus erythematosus Lupus Downloaded from lup. In contrast. neurologic disorder in 48. Patients and Method: The medical records of SLE patients had ever been hospitalized during 2011-2013 in National Taiwan University Hospital were reviewed. Age-specific Incidence Rates of Myocardial Infarction and Angina in Women with Systemic Lupus Erythematosus: Comparison with the Framingham Study. All subjects underwent a cardiac CT scan with assessment of the coronary artery calcium score (CAC).S. References 1 Manzi S. It also highlights the importance of prophylactic therapy in SLE patients with HBV infection.2. 22.3University of Southern Denmark Introduction: Patients with systemic lupus erythematosus (SLE) have an increased risk of cardiovascular disease (CVD).3% of the pts had criteria for antiphospholipid syndrome. References 1 Urowitz MB.2% pts. 3%). Isenberg DA.72.4%.3% cases. Odense University Hospital.sagepub.4) men with median age at the onset of disease of 45 years and mean SLEDAI 9.6% postmenopausal women. Person Chi-squre test was used to evaluate the diffierence of the liver prognosis. A034 LOWER PREVALENCE OF HEPATITIS B VIRUS INFECTION BUT WITH POORER LIVER PROGNOSIS IN SYSTEMIC LUPUS ERYTHEMATOSUS IN TAIWAN Lin T.P1.N.K. Aim: To clarify the prevalence and liver prognosis of HBV infection in SLE patients. Among the 24 patients. Diederichsen L.73-108. and Hsu P. This increased risk of CVD may be due to an increased prevalence of atherosclerosis. Division of Allergy. Djokovic A. In patients with SLE. among the SLE patients without HBV infection.9th European Lupus Meeting 462 A033 HIGH EXTENT OF CORONARY ARTERY CALCIUM AMONG DANISH PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS . Belgrade. renal damage (p¼0. CACS among SLE patients range from 09725. 2 Moss KE. which has been demonstrated to have major impact on morbidity and mortality. Farewell VT.3 years. There were 24 HBs-Ag positive patients. while among controls from 0-682. 145(5408–15.C. Thromboembolic event was cause of death in 33. Serbia Objective: To analyze risk factors for death outcome in SLE patients followed prospectively in a single center.A POPULATION-BASED STUDY Kay S. and same percentage of patients (11.317. Results: The SLE patients were 93% women.5% with cyclophosphamide and 98% of pts with antimalarics. After adjustment for age and sex.002). J Rheumatol 1997. and there were 37.9  14. p ¼ 0. The prevalance of HBV infection was calculated.002) and menopause (p¼0. Conclusion: Multi-organic involvement in older patients was associated with higher mortality in Serbian SLE patients attended in a tertiary care center. Gladman DD. high CACS (>400) were more frequent among SLE patients than controls (16% vs. High CACS is associated with age.1. Department of Internal Medicine. Average disease duration was 7. Methods: The study included 244 patients with SLE followed over a 20year period according to a standard protocol at the University Center ‘‘Bezˇanijska kosa’’. 80.com at Institute of Marine Biology of Crete (IMBC) on October 26. Median CACS did not differ between SLE patients and controls.1) died due sepsis and malignant diseases whereas in 7. hypertension and waist-hip ratio.3%. Poulsen M. Ioannou Y. Denmark. Iran 3Department of Biostatistics & Epidemiology.6. Porto 7Hospital Conde de Bertiandos. Terroso G. University of Oslo. At the end of 10 and 15 years it was similarly 90% whereas it dropped to 80% by 20 years. infections (29%) and cardiovascular diseases (23%) were the main causes of death.2 and Gran J. and to compare these with the general population.A2.5 1 Department of Rheumatology. Barcelos A. immediate and contributing causes of death. Tamaki C. Canas da Silva J.1. Fernandes S. Cardiovascular disease.8. Sayedbonakdar Z. Isfahan University of Medical Sciences.4.3. A POPULATION BASED STUDY Lerang K.1. 359 patients were alive at the end of follow-up. We believe the YPLL measurements supplements traditional methods of measuring mortality.1. Sequeira G. Oslo. The mean (SD) YPLL60 for females and males were 15 (11) and 8 (4) (p¼0. 85(3147–56. Duarte C. Faro 9Hospital de Aveiro.2. The patients were contacted to verify whether they are alive or not.7. Magder LS. 3 Rheumatology Research Unit.Reuma. Patients and methods: Multiple sources were used to identify 325 SLE patients within the city of Oslo during 1999-2009 who met 4 of the American College of Rheumatology (ACR) criteria.J.sagepub. Norway 3Institute of Clinical Medicine. Cerqueira M. References 1 Kasitanon N. 61: 409–413.3 1 Hospital Garcia de Orta. But. Gonc¸alves M. Gilboe I.4 and Smiley A. Almada. Aim: Characterization of the SLE Portuguese population included in Reuma. Active SLE disease is registered from medical record. Matinfar M. It is a web-based platform launched in September 2012 that simultaneously serves as a nationwide registry and as an electronic medical record.6. Ineˆs L. Central nervous system. Institute of basic medical science. Canha˜o H. 21. Lisboa 4 Hospitais da Universidade de Coimbra.6 years was the mean survival time.1. Major organ involvement including lupus nephritis (29%) and pulmonary hemorrhage (14%). age and ethnicity). Instituto de Medicina Molecular. CVD. et al.4. Conclusion: The study found an increased YPLL60 in SLE. Predictors of survival in systemic lupus erythematosus. The patients’ baseline characteristics including the clinical and laboratory manifestations recorded at the time of diagnosis were reviewed. The overall survival rate after 5. Norway Aim: To examine the rate and causes of premature death in a population-based cohort of Systemic Lupus Erythematosus (SLE). Isfahan University of Medical Sciences. Oslo University Hospital.4%. Silva C. Isfahan University of Medical Sciences. Norway 2Department of biostatistics. Rheumatol Int 2007. SLE patients had 259 years lost and 2241 years of observation before 60 years of age (116/1000) and the control population had 144 years lost and 11576 years of observation (12 /1000).4-23 years. Older age at onset ( 35 years).6. Iran 2Department of Internal Medicine. Thelle D.years was 93%. Survival study by organ disorders in 306 Japanese patients with systemic lupus erythematosus: Results from a single center. 27: 243–249. Lisboa 6Hospital de Sa˜o Joa˜o. The potential years of life lost (YPLL) was the sum of the numerical difference between a predetermined end-point-age (defined as 60 years old) and the age at death for deaths that occurred prior to that end point age. Costa L. Rikshospitalet. Bernardes M. Results: A total of 50 SLE patients died during the study period of whom 20 were under 60 years of age.P.C.9th European Lupus Meeting 463 attending a dedicated clinic for over two decades. Its 95 % CI was 20. Isfahan.3 and Santos M. Relevant factors of survival were explored through Cox regression analysis. 2Hospital de Santa Maria.PT/LES: THE PORTUGUESE LUPUS REGISTER Sousa S.5. Isfahan. Roma˜o V. Saber M. Miranda L.com at Institute of Marine Biology of Crete (IMBC) on October 26. Medicine 2006 May. Isfahan.2. Ann Rheum Dis 2002. 2014 . Isfahan.pt/LES Lupus Downloaded from lup.S.pt/LES. hematuria and pericarditis at the time SLE diagnosis were significantly associated with death by univariate analysis. Maracy M. Aim: This study was designed to determine the survival of SLE patients and its predictors in Iran Patients and Methods: 394 patients who were diagnosed with SLE and admitted to our hospital between 1985 and 2011 were included in a retrospective study. Raposo A.1.3.3. Iran 4 Department of Dermatology. Iran 5Isfahan.4. 3 Funauchi M. S. The YPLL (60) rate is found by dividing YPLL by years of observation for the population under age 60.5. T. Aveiro.9.7. Women and patients with kidney involvement lost on average 15 and 21 years respectively. Coimbra 5Instituto Portugueˆs de Reumatologia. seizure.1. A036 OUTCOME OF SYSTEMIC LUPUS ERYTHEMATOSUS IN IRAN Fatemi A. only pericarditis and seizure kept their significant effects on survival after multivariate analysis.J.08) and for patients with and without lupus nephritis 21 (10) and 8 (6) (p¼0. The Years of Potential Life Loss before 60 years of age (YPLL60) and causes of death of these patients were examined and compared to a matched control population.A. Portugal Introduction: The Rheumatic Diseases Register from the Portuguese Society of Rheumatology has expanded its coverage to Systemic Lupus Erythematosus (SLE) . Pereira da Silva J. Results: Female/male ratio was 8. CNS. Lisboa.2. University of Oslo.5. A038 REUMA. M. The analysis includes underlying. Silva J. Isfahan University of Medical Sciences.1. Its aim is to register all patients with SLE and follow them up in a standard manner in order to improve the monitoring and clinical care for patients with SLE while increasing the knowledge of this disease. Shimadzu H. Figure 3 Annual years of potential life loss before 60 years of age (YPLL60) related to specific causes of death per 1000 SLE patients and their control subjects (matched for sex. Ponte de Lima 8Hospital de Faro. Conclusion: Our study showed comparable survival rate of patients with SLE in Iran to that in developed countries.3 1 Department of Rheumatology.1.2. The causes of mortality were extracted as well. Iran Introduction: No study about mortality in systemic lupus erythematosus (SLE) in Iran has been yet published in Pubmed.01) Figure 1 rank the attribution of causes of death in SLE in consecutive order. Petri M. A037 PREMATURE DEATH IN SYSTEMIC LUPUS ERYTHEMATOSUS MEASURED BY YEARS OF POTENTIAL LIFE LOSS BEFORE 60 YEARS OF AGE. Aim: We investigated the causes of death.3% with antimalarials. Zdrenghea M. Boumpas D. Greece 3Rheumatologist 4Department of Social Medicine.038) were independent poor prognostic factors which influenced survival. and the 10-year survival was 92%. treatment modalities. Mamoulaki M.5/100000 (females. with the ultimate objective of improving patient care and simultaneously scientific research in the field of SLE.1. 28. etiopathogenesis and natural history of complex diseases such as systemic lupus erythematosus (SLE). Heraklion. disease activity and damage scores were analysed in 117 SLE patients followed-up between 1990 and 2013. autoimmune hemolytic anemia.008).25.6 years) diagnosed with SLE between 20032013. treatments and responses to various treatment modalities were recorded.1%. respectively. neuropsychiatric involvement (p¼0.71.7/100000. genetically homogeneous population of 0. Results: The mean annual incidence of SLE was 4.9 years. Major organ involvement was present in following percentages: neurologic involvement. 95%. 53% of the Lupus Downloaded from lup. cumulative clinical features.9th European Lupus Meeting 464 Patients and methods: SLE patients registered in the Reuma. Rinzis M.015). mean age: 38. p¼0. Melissourgaki M. 300000F).2.2. p¼0. when available.sagepub. Division of Rheumatology.6%. Thirteeen SLE patients (10 females. In our series. initially active disease and autoimmune hemolytic anemia were poor prognostic factors. Chatzi L.3% vs. being the most frequent hypertension (31.5%.6M inhabitants.88. mortality in systemic lupus erythematosus (SLE) is still high.T.pt/LES until December 2013 are described. The 5-year survival was 95%. et al. Causes of death were defined on the basis of clinical data and.9% of other races. majority of patients were females. pleural involvement.N.8. Results: 1510 patients were included. According to Kaplan-Meier survival analysis.1. Edirne.3. thyroid diseases (10. CLINICAL FEATURES AND ENVIRONMENTAL FACTORS IN A GENETICALLY HOMOGENOUS.23. SLEDAI-2K at last visit was in average 2. 87%.B.7/100000. Trakya University Medical Faculty. showing greater mortality in patients with renal involvement.014). renal involvement. SLE-related deaths occured due to active disease (pulmonary or cerebral hemorrhage) in a third of patients and due to end stage organ failure (renal or cardiac) in the rest. p¼0.pt is a very useful tool that allows a more efficient patient follow-up. and overall survival rates at 5. hemolytic anemia (OR: 4.2. 96.3.1. 94.4% vs. Katzakis P. usage of cyclophosphamide (5-year: 87. Greece Introduction: Crete is the third largest and southernmost island in the Mediterranean with a relatively stable.005). associated antiphospholipid syndrome (p¼0.5 and Bertsias G. thus offering the opportunity to study the prevalence. treatment responses and prognosis of systemic lupus erythematosus (SLE) patients diagnosed at our center in Thrace region of Turkey. Clinical Immunology and Allergy. Do¨nmez S. while demise by catastrophic APS or cancer occured predominantly afterwards.1.003). Secondary Sjo¨gren syndrome and antiphospholipid syndrome were documented in 10. respectively.1. having pleural involvement (5-year: 95.18. highlighting the need to reduce damage accrual and improve the management of catastrophic APS in SLE patients. SLICC/ACR damage index was in average 0. Conclusions: Reuma.3. Cluj. clinical determinants of survival and survival rates in a longitudinal cohort of hospitalized SLE patients.8. A039 SURVIVAL ANALYSIS AND CAUSES OF MORTALITY AMONG INPATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS IN A ROMANIAN TERTIARY CARE HOSPITAL OVER 20 YEARS Pamfil C. infections (13%) and cancer (13%). The multivariate analysis of risk factors identified a predictive model consisting in a cumulative damage score 3 (p¼0. About 50% of the patients were ever treated with corticosteroids and 63.5% are Caucasian. We also estimated prevalence and incidence of SLE in our region.018). Fanouriakis A. Co-morbid conditions were recorded in 641 patients. The survival curves of patients with and without lupus nephritis diverged in the first 20 years of disease. Athens. 19M. In Crete. Greece 2Institute of Molecular Biology-Biotechnology. poor prognostic factors were being male (5-year: 77% vs. University of Crete.1. Calayır G. however.4. and trombocytopenia. Flestea A.011). Cluj 3Babes-Bolyai University.and 10-years were 93% and 88%.1.2.8%. Department of Internal Medicine. Cluj 2Emergency Clinical County Hospital. males.1. 2014 . Spyrou G.05). hematological and musculoskeletal.0. Popov I. and Mengu¨s¸ C¸. The overall prevalence of SLE was 45. males.. Results: A total of 15 (80% female) out of 117 patients died. Tzanakakis M.82:299-308. p¼0.1. Stroe I..045).1/100000).1 1 Iuliu Hatieganu University.711. and standardized data collection and analysis. 4.7%.6%. and an initially high SLEDAI score (>6) (5-year: 89% vs 97. The mean disease duration was 13.4% vs.9 years and the most prevalent manifestations were mucocutaneous.2.5%). Survival was similar to data from western countries. Clinical features.2.6. p¼0.6% black and 0. p¼0. FORTH. Kabouraki E. p¼0. Deaths due to infections and active disease prevailed in the first five years of disease.8%) and diabetes (7.1. The majority (92%) are women with an average age of 47.533.1. Romania Introduction: Despite progress in management. Medicine 2003.6% of patients. and low C3 level at the time of initial diagnosis (OR: 5. References 1 Cervera R. SLErelated deaths were the main cause of demise (40%). Conclusions: Our study demonstrated that the annual incidence and prevalence of SLE in Thrace region of Turkey were quite similar to western data. Conclusion: Long-term prognosis remains poor in patients with severe disease and associated antiphospholipid syndrome. having renal involvement (5-year: 88. Our hospital has been the only tertiary referral center for rheumatological diseases for a mixed rural and urban population of 616000 people for >16 years (316000M.2%).1 and Rednic S.05). 3 males) died at a median follow-up of 49 months. Kallitsakis I. A041 THE CRETAN LUPUS COHORT ‘‘LETO’’: PREVALENCE.1. 9. Greece 5University of Athens. Turkey Introduction/Aim: We evaluated the clinical features. Patients and Methods: We retrospectively evaluated 279 patients (260F. pleural involvement (OR: 6.2.6/100000). Repa A. 20. Sidiropoulos P.004). The global mortality was 13%. p¼0.com at Institute of Marine Biology of Crete (IMBC) on October 26.2 1 Rheumatology.2%.714. 98. Renal involvement. Felea I. University of Crete.04) and advanced age (p¼0.1. Candrea E.6% and 7. 14. postmortem examination. lupus nephritis was not a major determinant for survival upon univariate analysis. Patients and Methods: Demographic data.2. p¼0. Survival rates were calculated by the Kaplan-Meier method and log-rank test. Damian L. followed by catastrophic APS (34%). renal involvement (OR: 4. MIXED URBAN AND RURAL SOUTH EUROPEAN POPULATION Gergianaki I.86.1.3/100000 (females. According to Cox regression analysis. A040 THE CLINICAL FEATURES OF SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS FOLLOWED UP AT A SINGLE CENTER IN THRACE REGION OF TURKEY Pamuk O. infectious diseases (24. The most common features were mucocutaneus (96.1. Italy Introduction: Lupus nephritis (LN) is a common manifestation of Systemic Lupus Erythematosus (SLE). d’Ascanio A. Sex-ratio was 3. and clinical characteristics (including disease activity. Outcome was classified as: remission -no clinical signs of disease and with normal lab parameters during six months. and in determining socio-demographic. Aim: To analyze the mortality profile related to SLE in France.3%). musculoskeletal (93.5  17. Anti dsDNA in 81. age. aggressive immunosuppressive therapy has considerably improved the patient’s survival but long-term renal outcome is still an open issue.8 / 106 people) and the mean age of death was earlier. 2014 .6%).9%) and hematological (90. Two patients died from secondary antiphospholipide syndrome after 10 and 14 yrs.3.7%).5%). and quality of life scores) are electronically registered using structured forms.3% of patients. sunlight exposure. All hospital records were reviewed and all in. France 2Ce´piDc. Bombardieri S. Results: A total of 635 SLE patients aged 15 years who reside permanently on Crete were identified. To monitor environmental exposures in association with disease outcomes.1.1.2%). The diagnosis was established using ACR updated criteria (1997). For SLE as an underlying cause.4%) pts.192. Belgrade. serves as the referral center for patients with rheumatic diseases in Crete and has an extensive network that captures primary and secondary care including private rheumatologists. Aim: To describe experience in the treatment and outcome of disease in children and adolescents with jSLE. Susic G2 and Novakovic D.1.) Period from first symptoms to established diagnosis was 3. both at national and regional level. University of Crete. 8% in suburban (10. The observed/ expected death ratio (O/E ratio) was calculated for the main causes of death. micophenolat-mofetil (21. Paris. Querci F.1. the most common underlying causes of death were cardiovascular diseases (35. but was <1 for neoplams.2. For SLE as a non-underlying cause of death. azathiophrine (48.9%) and infectious diseases (10. adherence to Mediterranean diet.8%). Jougla E. Rheumatologists working in private practice were also contacted to recruit additional patients. active disease in 38% of patients and three pts died. Results: Thirty seven patients (36 f.5% of children.3 years. satisfactory control of disease -one unlimited sign of disease. flares. Corticosteroid treatment was given in all patients as prednisone (100%) or methylprednisolone pulses (58. Patients and Methods: The Department of Rheumatology. Tavoni A. INSTITUTE OF RHEUMATOLOGY BELGRADE. past medical history. and one patient died from multiple brain abscesses’ during sepsis.7 years (range 7-19 yrs.5%).1. mixed urban and rural population in a setting that combines primary. inserm.2 and Harle J. In this study we aimed at describing the very long-term renal outcome of a monocentric cohort of SLE patients.000 dwellers). sex-ratio. Conclusion: Cardiovascular diseases are the most important cause of death associated to SLE in France with a significant excess of mortality compared to general population. tobacco and alcohol use. The mean standardized mortality rate was 3. Conclusion: Satisfactory controlled disease was in half of the patients. The average age at the time of diagnosis is 41 (15) years (range 9-73) and female:male ratio 6.1%. ANA was positive in 97. satisfactory control in 19 (57. and death.com at Institute of Marine Biology of Crete (IMBC) on October 26. affecting up to 60% of patients.3% of patients.6%) and cyclosporine (3. Anti Sm in 40 % and APA were positive in 37. neoplasms (13. Socio-demographic.R.5 yrs (1 month-30 yrs). In overseas departments. data collected in the French Epidemiological Center for the Medical Causes of Death (CepiDc) database and corresponding to death certificates of adults (n¼1593) on which SLE was listed as an underlying (n¼637) or nonunderlying cause (n¼956) of death were analyzed using multiple causeof-death analysis. A044 VERY LONG-TERM RENAL OUTCOME OF LUPUS NEPHRITIS: A SINGLE CENTRE EXPERIENCE Tani C. Results: Mean age at death was 63.and outpatients diagnosed with SLE (4 ACR criteria) were prospectively evaluated. Outcome of jSLE was: remission in 1 patient (3%). the main non-underlying causes of death were cardiovascular diseases (49. Drugs used were: antimalarics in 97. corresponding to a disease prevalence of 123 per 100.9%).2 per 106 people. Serbia 3Clinical Center Banja Luka.196.2:1. Gender. damage. 7 months after diagnosis of jSLE. Patients and Methods: The medical records of patients with jSLE treated in the period 2001-2011 were reviewed. follow up of out-patients and especially the treatment with new therapeutic approach.1.9th European Lupus Meeting 465 inhabitants reside in rural.3%.2%. Stanimirovic B. Neri R.000-15. Banja Luka. Bosnia and Herzegovina Introduction: Juvenile systemic lupus erythematosus (jSLE) is autoimmune disease with 15-20% onset in childhood and adolescent age. School of Medicine 2Institute of Rheumatology. Lupus nephritis occurred in 45.5%) and renal failure (23.sagepub. The mean age at disease onset was 15.5. environmental and other factors that affect its natural history. A042 TREATMENT AND OUTCOME OF JUVENILE SYSTEMIC LUPUS ERYTHEMATOSUS IN PEDIATRIC UNITE. are instrumental in the description of complex disease such as SLE.1 1 La Conception hospital. Lupus Downloaded from lup. Vagnani S. Aim: To establish a Cretan cohort of SLE patients and register their demographic and clinical characteristics. department of internal medicine. secondary and tertiary care.1.1.000 dwellers) and 39% in urban areas (>10.2 1 University of Belgrade. SERBIA Stojanovic R. The overall death O/E ratio was > 1 for infectious and cardiovascular diseases and renal failure (especially for people < 40 years of age for the 2 last causes). active disease -two or more signs of disease. Urbanization. the standardized mortality rate was higher (10. Department of Clinical and Experimental Medicine.1.1 and Mosca M.9%). CNS manifestations in 51. A043 TRENDS OF MORTALITY ASSOCIATED TO SYSTEMIC LUPUS ERYTHEMATOSUS IN FRANCE: A MULTIPLE CAUSEOF-DEATH ANALYSIS Chiche L. as well as major stressful events are monitored using validated questionnaires and their effects on disease outcomes. 1m) with jSLE were reviewed. The duration of jSLE was 5. Aix-Marseille University. Eighty eight (14%) patients have biopsy-proven lupus nephritis and 46 patients (7%) have neuropsychiatric lupus.000. and active disease in 14 (42. standardized mortality rates as well as the respective weight and frequency of the various causes of death were assessed. Carli L. It is therefore essential to improve therapeutic and preventive interventions in this population and improve survival in SLE. France Introduction: The overall mortality rate of Systemic Lupus Erythematosus (SLE) has improved significantly over the past 50 years but is still high as compared with the general population. such as flares and severe manifestations. Conclusion: Longitudinal studies with genetically homogenous. Marseilles. Thomas G. University of Pisa.8%).1 1 Rheumatology Unit. It is necessary to improve diagnostic methods. Patients and Methods: For the 2000-2009 period.2 months. is prospectively examined. Despite obvious differences in therapies and management over the years. In 2012 the SLICC group proposed an alternative classification for SLE.1. III 10. References 1 Petri M. Alder Hey Children’s Hospital. 279 patients fulfilled 4ACR criteria and 74 had 3 ACR criteria at time of diagnosis. Direct immunofluorescence examination revealed antibodies IgG in the epidermis. Clinical characteristics observed in JSLE patients with biopsy proven nephritis Biopsy JSLE without biopsy confirmed LN confirmed LN 72 281 12. Gordon C. myelitis. more than 30% of patients reached a poor outcome after a mean of 16 years since LN onset.4g0. neurological manifestations) and early diagnosis is therefore important. 78 (69%) for anti-dsDNA and 50 (44%) for anti.32 years. An histological LN class diagnosis was available in 105 patients (II:7. The cumulative GC dosage was 31.1 (min 0. Results: Among the 250 patients with LN of our cohort.. 2014 . Beresford M. Merrill JT.1. Compared to those without biopsy-proven LN. The patient was treated by Encorton 30mg for eight month.4%) had been transplanted. min 10-max 36). Patients and Methods: Data was available for 353 patients at diagnosis and latest follow up (median 3.9th European Lupus Meeting 466 Methods: In this retrospective observational study. thus fulfilling the SLICC criteria in advance of meeting 4ACR criteria. all patients were positive for ANA.8%) had an end stage renal disease and 5 (4. 60(53%) Azatioprine.2. UK 2Institute of Translational Medicine (Child Health). only patients followed for at least 10 years at our centre were included in the analysis.1. a large national inception cohort. Filipowska B. 113 had at least 10 years of fu (median since renal disease onset: 16. et al. 39 patients (34. Arthritis and rheumatism 2012. enrols patients 18 years old with 4 American College of Rheumatology (ACR) criteria and those with probable evolving JSLE (<3 ACR criteria). Discussion: In this retrospective cohort.8: 1 8% (23/281) 12% (31/266) 43% (93/217) p ¼0. 38 (33%) Cyclosporine. 26 (23%) Mycophenolate Mophetile.8: 1 Neurological Involvement 19% (ACR Definition)y (14/72) Neurological Involvement 25% (SLICC Criteria Definition)z (17/68) Low baseline C3 77% (41/53) y 5.004 p ¼0. Patiens and methods: We report a case of a 57-year-old female patient suffering from subacute cutaneous systemic lupus erythematosus with a one year history. but not the face. Indirect immunofluorescence examination revealed antinuclear antibodies (titer 1:640).006 Recorded as of latest visit Recorded as of latest visit: SLICC Criteria for Neurologic Involvement(1) (seizures. trunk.. Diagnostic. IV: 84. SLICC criteria may improve sensitivity for diagnosis of JSLE.003 p ¼0. Those with biopsy-proven LN are more likely to have severe disease (e. She is staying under clinical observation. UK Introduction: Juvenile-onset SLE (JSLE) is reported to feature more frequent renal involvement than adult-onset SLE. A046 APPLICATION OF THE SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS CLASSIFICATION (SLICC) CRTIERIA TO JUVENILE-ONSET SYSTEMIC LUPUS ERYHTEMTAOSUS NEPHRITIS COHORT Morgan TA. Results: Histopathological examination taken from skin lesions revealed perivascular lymphocytic infiltrates with intercellular edema. Smith E.5%) reached a poor renal outcome: 24 (21.sagepub.7%) received Rituximab.8 12. were significantly more frequent in patients with biopsy-proven LN at first presentation (see Table 1).2%) had a chronic renal failure. Table 1. Four patients still do not meet the ACR criteria at latest follow up.711 years and the mean time between SLE diagnosis and LN resulted 4. as well as the presence of low C3 concentrations. despite treatment. Skin lesions were localized on the limbs.622. it should be taken into account in the differentiation of these two skin diseases. Alarcon GS. Results: 83/353 patients (24%) had a renal biopsy performed and 72/ 353 (20%) had biopsy-proven LN. biopsy-confirmed nephritis compatible with SLE in the presence of ANAs or anti-dsDNA antibodies is sufficient for diagnosing SLE. 64(82677–86. thickening of the keratin and parakeratin.2.2 years). Aim:To identify amongst JSLE patients with biopsy-proven lupus nephritis (LN) those meeting SLICC classification criteria but not meeting ACR criteria for SLE.4. Weryn´ska-Kalemba M. 10 (8. the median age at renal disease onset was 30. the median age of the cohort was 4611 years (range 23-82). 13 (11%) were also treated with plasma exchange while only 2 (1. Fortin PR. Cumulatively. Zawadzinska K.1. V: 3). Clinical Department of Internal Medicine. SLE patients with evidence of LN were evaluated.com at Institute of Marine Biology of Crete (IMBC) on October 26. The UK JSLE Cohort Study. Local and general treatment had improved clinical status of the patient’s skin. All Caucasian. Liverpool. At our last observation. 10/72 (14%) patients with features of LN on biopsy had <4ACR criteria but were ANA and/or dsDNA positive.7 g19 (min 5 max 140 g).g. 81% of patients received Cyclophosphamide. Therefore.1. our data might offer a real-life representation of longstanding LN and of unmet needs on the treatment of this severe SLE manifestation. scalp and vulva. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Lloyd O.(1) In these criteria. peripheral or cranial neuropathy or acute confusional state) z Lupus Downloaded from lup. Orbai AM. Conclusions: Application of SLICC classification criteria to patients in whom renal biopsies were undertaken increased the chance of a diagnosis compared to using ACR criteria alone. Skin changes were characterized by erythema and infiltration. mononeuritis multiplex. which fluorescence with HEP-2 cells was granular. neuropsychiatric lupus using both ACR and SLICC criteria. Heaf E. Conclusion: Based on the analyzed case we would like to present psoriatic form of SCLE. the mean creatinine value resulted 1.56 years.2 and on behalf of the UK JSLE Study Group 1 Paediatric Rheumatology Department.W. enabling earlier diagnosis within a paediatric population. Zabrze. There were covered by psoriasiform plaques. Characteristically the lesions appear in sun-exposed areas such as the neckline or the forearms. Poland Introduction: Subacute cutaneous lupus erythematosus (SCLE) is a clinically distinct subset of cases of lupus erythematosus that is most often present in white women aged 20 to 50. University of Liverpool.7 (min0-max 4) being 3 patients with nephrotic range proteinuria.. Dermatology and Allergology.max 11) and the mean 24 hours proteinuria was 0. classification and outcome criteria A045 A CASE OF FEMALE PATIENT WITH PSORIATIC FORM SUBCUTANEOUS LUPUS ERYTHEMATOSUS Filipowska-Gron´ska A.phospholipids. psychosis.2 Number Patients Mean Age at diagnosis (Years) Female: Male Ratio 5. which clinically resembles psoriasis. 19 (17%) IvIg. and Jarzab J. At the time of the biopsy. 2%).411.2 14. as the Quality of Life (QoL). Rheumatol 2010. 45.65 3.1.3%). Report of a Task Force of the EULAR Standing Committee for International Clinical Studies Including Therapeutics.039).7. characterized by variable presentations and a relapsing-remitting nature. 94. Ch. Patients and methods: The study cohort included patients from Moldova Lupus Study (MoLuStudy) diagnosed with SLE fulfilling at least 4 of the 1997.S. mean age SD 39. Vetrila S. Cebanu M. The disease activity was appreciated by SLEDAI and SLAM.8) and younger at diagnosis (31.1. Revenco N.06.0 and SLAM . 6 12. Aim: To assess the QoL and to establish its correlation with the disease activity and global assessments in lupus patients Patients and methods: Cross-sectional study including patients fulfilling the SLICC 2012 SLE criteria. mucocutaneous (22.’’. We established a significant correlation between the onset of symptoms until the first medical contact with disease activity (r¼ 0. those with lupus nephritis and on immunosuppressive treatment are at risk of flares whilst hydroxychloroquine reduces flare rates. et al. mean disease duration was SD 108 12. 99 female (91. neuropsychiatric (11.9 months.47 8.sagepub.. Results: There were 78 patients in the cohort. other outcome measurements.4%. The Lupus Downloaded from lup.4%. giving a flare rate of 15. 100% females.17%).2%).2%). the data on the relationship between the QoL and the dis ease activity are contradictory [2. Causes of Death and Prognostic Factors in Thai Patients with SLE. At each monthly visit. the mean time from the FMC to diagnosis and start of treatment of SLE was 3.213. fever and immunological or hematological abnormalities).7%).6%. 67.5%). arthralgia. demographics and use of hydroxychloroquine in patients with and without flares.4%). East Lancashire Hospitals NHS Trust. 53. current treatment and BILAG-2004 scores were recorded. use of corticosteroids and immunosupressive drugs [2].21.12. Results: We examinated 108 patients. et al.9th European Lupus Meeting 467 A047 FACTORS ASSOCIATED WITH TIME TO DIAGNOSIS IN SYSTEMIC LUPUS ERYTHEMATOSUS IN MOLUSTUDY Mazur-Nicorici L. ‘‘Numerical scoring for the BILAG-2004 index. MazurNicorici L.7 months. constitutional (11. The disease activity by SLEDAI was 12. United Kingdom Introduction: SLE is a complex disease. Asian/Pakistani/Indian (33.1. aiming to identify predictors of flares.71.11: 1372–1379. A049 WITHDRAWN A050 IS THE QUALITY OF LIFE INFLUENCED BY THE DISEASE ACTIVITY IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS? Sadovici V. Ethnicity in the flare and non-flare patients was: White/Caucasian (66. ACR revised criteria. Ch.cutaneus manifestation. Germany Introduction: Patients with systemic lupus erythematosus (SLE) improved their 10-year survival during the last 40 years [1]. Rheumatol 2003. Baerwald2. The mean time duration from the (OS) to first medical contact (FMC) was 2. 3]. demographic data. Blackburn. general assessments by PGA and MGDA. musculoskeletal (20. Early onset systemic lupus erythematosus: differential diagnoses. There were no differences between the two groups with regards to gender or duration of SLE. and Teh L. References 1 Gordon C. gastroenterology (9%. 3713. and treatment options. 18 developed a new A or B BILAG-2004 flare in 18 months. McElhone K.1 1 SUMPh Nicolae Testemitanu. renal (4. The SLE disease activity was determinated using the SLEDAI and SLAM scores. ‘‘Identification of clinical predictors of flare in systemic lupus erythematosus patients: a 24-month prospective cohort study.7%. At baseline. 30(2275–83. Descriptive statistics and student t-tests were used to compare the groups.7% of the patients who flared had at least one re-flare. et al.3. 2 Hedrich CM. 51. All patients completed the Sf-8 questionnaire. Moldova 2Leipzig University. et al. Asian Pacific Journal of Allery and Immunology 2002. 50% of flare patients were not on hydroxychloroquine compared to 24% of non-flare patients (P¼0..1: 85–89. There were 44 baseline and 65 re-flare episodes. DEMOGRAPHICS AND USE OF HYDROXYCHLOROQUINE IN PATIENTS WITH AND WITHOUT FLARES Makaronidis J.9). Conclusions: Younger patients with a younger age of onset of SLE tended to flare.5%. Results: We examined 30 patients. et al.1 1 SUMPh ‘Nicolae Testemitanu’. It has been suggested that younger patients.23.’’.6 years.points. 2 Ineˆs. Aim: This observational cohort study compared clinical features. Luı´ s. respectively. ‘‘Definition and treatment of lupus flares measured by the BILAG index. et al.6.com at Institute of Marine Biology of Crete (IMBC) on October 26. 3 Yee. Patients and Methods: A cohort of patients with SLE under the care of one Rheumatology department was observed for 18 months. 20: 85–91.2 10.1 and Mazur M. In the literature. This improvement was related to a better undesrtanding in the immunopathogenesis of SLE.5%. BILAG-2004 scores and treatment changes were collected.5%) and Black African/Carribean (0%.3%. References 1 Kasitanon N.9: 1665–1669. Patients with new A or B flares using the BILAG-2004 index were followed up monthly for 9 months. therefore.9.513.1. Republic of Moldova 2Leipzig University. The patients who flared were younger (mean SD.’’.1 years (well-described signs or symptoms that were compatible with the disease. Germany Introduction: During the past decades it was established an increase of the survival rate from a 5 –year in the 1950s to a 10 years survival rate of 90% in 1990s [1].’’.9 years.5%. A048 FLARE RATES IN SYSTEMIC LUPUS ERYTHEMATOSUS: A LONGITUDINAL COHORT COMPARING CLINICAL FEATURES.. Aim: To determining the time from onset of symptoms (OS) until the diagnosis of SLE patients in Republic of Moldova. 42. Clin Rheumatol 2011 Feb. psihoneurological involvement. Conclusion: Increase the time from first symptoms to initiation of treatment involves high activity and significant correlation with low level of education. early detection of cases. Cebanu M. Chee-Seng.432) and (r¼0. A larger proportion of patients who did not flare were on hydroxychloroquine. estimating physical and mental component summaries (PCS and MCS).1.392) and was appreciated a good correlation between speedy addressing patient with intermediate and high level of education (r¼ 0. 2014 .1 and Mazur M. mean disease duration SD 5.1. mean age SD 40. Rheumatol 2014. Sadovici V.1%).2: 195–205. clinical presentation.8. Ann Rheum Dis 2008.7%). ‘‘EULAR recommendations for the management of systemic lupus erythematosus.3%) and haematology (0%.1.451).1. gained importance [2]. Pasali M. The frequency of the systems affected at the initial and subsequent flares were: cardiorespiratory (20.4%. 4 Bertsias G. 49. Baerwald2. The patients were separated into two groups (flare or non-flare).5.24. 1.787 0.4.832). Their relationship with the underlying disease could be difficult to assess especially for mild. including a weighted score of 4 different items: ‘onset time’ of the NP event. Presented at: ACR 2012 Annual Meeting. P937: Changes in Ten Year Survival Among SLE Patients At an Academic Center in North America (1970-2011). A053 RELATIONSHIPS BETWEEN MINOR NEUROPSYCHIATRIC EVENTS AND SYSTEMIC LUPUS ERYTHEMATOSUS: PERFORMANCE OF AN ATTRIBUTION MODEL Bortoluzzi A.1 1 Section of Haematology and Rheumatology. as shown in the table. PCS MCS Correlation Coefficient p Correlation Coefficient p SLEDAI SLAM PGA MGDA -0. 2 failed the transplant and currently on hemodialysis.5705 -0.1 and Al-Mayouf S.823 0. Performance was evaluated by the Area Under the ROC Curve (AUC).0 and 39. and standardised coefficients. 4 patients underwent renal transplant. Quality-of-Life Measurements Versus Disease Activity in Systemic Lupus Erythematosus.9th European Lupus Meeting 468 disease activity by SLEDAI and SLAM was 7. with a lower contribution for ‘onset time’ 2. Expert opinion was set as reference standard of the attribution of NP event to SLE.1 and Govoni M. A051 COPYRIGHT PROTECTED.27.0034 -0.18 (1. and co. Six patients had got work. Central nervous involvement was found in 16 patients in the form of seizure disorder (6 patients).sagepub. chorea (3 patients) and cerebrovascular accident (3 patients). with a median age at first NP event of 38 years. Eight patients got married and 5 of them have children Conclusions: Our cohort indicates that the outcome of adult patients with childhood onset SLE was satisfactory and comparable to previous reports.182 0. The PCS and MCS were 43.0024 -0. Rheumatol 2004.com at Institute of Marine Biology of Crete (IMBC) on October 26. PGA and MGDA represented 42.0025 -0. Milan.112 0. Petri M.87. The relationshio between disease activity and quality of life in systemic lupus erythematosus.7586 References 1 Merola JF. The relationship between the attribution model and the reference diagnosis was evaluated by logistic models. University of Ferrara and Azienda Ospedaliero-Universitaria Sant’Anna di Cona.1.631. Padovan M.830 0.0008 -0. the QoL being low by both components.837 0. Riyadh Objective:To describe the social. diffuse and nonspecific NP syndromes such as. The most important items for correct classification was the ‘confounding factor’ sub-score OR (95%CI)/stand.22-3.3116 -0. Forty patients (83%) had renal involvement.754 (0. Conclusion: Despite expert’s opinion remains the gold standard. NOT FOR PUBLICATION A052 OUTCOME OF ADULTS WITH CHILDHOOD ONSET SYSTEMIC LUPUS ERYTHEMATOSUS IN SAUDI ARABIA Al Hamzi H. 2 Kiani A. 3 Khanna S. and results presented as odds ratio (OR) and 95% confidence intervals (CI). with mean of damage index of 2 (0-7). 15.75.51-4.71 (1.6 and 46. 31 mild depression. Al Shaikh A. associations as suggested by the 1999 ACR criteria). and ‘additional factors’ 2.2 Adult Rheumatology1. presence of specific NP events according to Aniala. mild depression and anxiety. Ferrara 2 Epidemiology Unit. Methods: All adult patients with childhood onset SLE treated between 1990 and 2013 at King Faisal Specialist Hospital and Research Centre (KFSH-RC). We established a close significant negative correlation between PCS and disease activity. presence of ‘favouring factors’ (general and disease specific).25%). Conclusion: The Physical Component Status of the QoL has a high indirect correlation with the disease activity and global assessments in patients with Systemic Lupus Erythematosus.357 0. Patients and Methods: NP events were defined according to the 1999 ACR case definitions and re-assessed using a previously developed and validated attribution model. Results: A total of 126 patients with a diagnosis of SLE according to the revised ACR classification criteria with one NP minor event (86 headache. Pediatric Rheumatology2 King Faisal Specialist Hospital and Research Center.326.8. coeff. Curr Rheumatol Rep 2010. 43:1536. employment and long term clinical outcomes of adults with childhood onset systemic lupus erythematosus (SLE) in Saudi cohort. 21 of them started college. 9 anxiety) were included: M/F 5/121 females.N. Results: 48 patients (45 female) were included. The MCS did not correlate with disease activity scores.6882 -0. Aim: To test the performance of an attribution model applied to mild NP events in a large mono centric cohort of SLE patients. Riyadh and continued to be followed at KFSH-RC are included.59-70. mainly due to infection.85)/0. Social. Lupus Downloaded from lup.e. 2014 . presence of non-SLE ‘confounding factors’ (i.03)/1.M.2.. Scire` CA.61. There were 3 deaths related to SLE (6. Table 1.675-0. The long-term outcome measured by SLE Disease Activity and Damage Indices at last follow up visit and death related to SLE were determined. using an assignment algorithm may improve the correct attribution of minor NP events to SLE. headache. Italian Society of Rheumatology.92)/0. Nov.130 0. Alhaymouni B.86 (3.710 and 7. 24 (50%) patients were found to have active disease (SLEDAI > 4).913. The overall performance of the model was good (AUC 0. Washington. The mean age was 23. educational and employment history and quality of life were obtained via personal or phone interviews. educational. 10-14. At last follow up visit. respectively. Forty three patients had completed secondary high school.6 þ 4 years while the mean disease duration was 15þ 4 years. 7 (15%) of them progressed end stage renal disease. Italy Introduction: Neuropsychiatric (NP) involvement in Systemic Lupus Erythematosus (SLE) presents with a variety of neurologic and psychiatric syndromes.1.29.. 12:250.4. Dermatology and Alergology in Zabrze.0vs24. Werynska-Kalemba M. phosphorus and PTH.Italy)... hematological and rheumatological consultation. Patients and methods: 34SLE women were enrolled. A group of 18pts (group S) were given ‘‘standard’’ regimen of supplementation (Cholecalcipherol 25. The most often is presented in white women aged 15 to 40.8 p¼0. two histopathology examination of skin section. exfoliative medicaments. The patient have had numerous blood analysis..more data will come from the second year of the study.There were 3 cases of transitory mild hypercalciuria (2 in I.sagepub.The pts had clinically quiescent disease (median SLEDAI 2 in S. Conclusions: Based on the analysis case we would like to present clinical difficulties that can occur during diagnosing psoriasis-like SCLE. Andreoli L. No significant differences upon season of enrollment. Pagacz J. then 50. Aim: To evaluate at 12months of follow-up (T12) efficacy.Brescia.p.9th European Lupus Meeting 469 Clinical features. 4 in I).C4. A055 A PROSPECTIVE STUDY IN PREMENOPAUSAL WOMEN WITH SYSTEMIC LUPUS ERYTHEMATOSUS SUPPLEMENTED WITH TWO DIFFERENT REGIMES OF VITAMIN D: EFFICACY AND SAFETY AT 12 MONTHS OF FOLLOW-UP Dall’Ara F. In the beging of observation only psoriasis like skin lesion were observed.LIKE SUBACUTE CUTANEOUS LUPUS ERYTHEMATOSUS Krajewska A. Lupus Downloaded from lup. and Tincani A.000UIonce/month). 2014 . In treatment topical steroides. including lues tests. After 3.02). Piva N. Piantoni S.000UI bolus. while at T12 sufficient pts were 28% in S and 75% in I (p¼0. Zawadzinska K. SSB and Ro-52 autoantybiodies. Patients and methods: 63-years female patient hospitalizated repeatedly in Chair and Clinical Department of Internal Diseases. Results: During hospitalization in 2011 in the first histopathology (HP) examination diagnosed ezgema with accompanying positive ANA antybodies (1:640). Spedali Civili and University of Brescia.No statistically significant variation in the titers of anti ds-DNA and in the levels of C3. Dermatology and Alergology in Zabrze beetwen 2011 and 2013. The patient is steal staying under clinical observation. chest X-rey. laryngological.1 in S). Coexistence of leukopenia was observed. Rheumatology and Clinical Immunology. et al. She also has cardiac.. DIF – common autoantybodies. safety and the effects on SLE disease activity of an oral Cholecalcipherol supplementation given with 2different regimens. and Jarzab J.com at Institute of Marine Biology of Crete (IMBC) on October 26.No particular effects on serological SLE parameters was noted. renal cyst were diagnosed. Then typical Ðbutterfly’’ face rush. Results: At baseline(T0) there was no significant difference in VitD levels in the 2groups. No significant variations in the levels of calcium. References 1 Cutolo M. Generally skin lesions disappear spontaneously during 6 month period. Vitamin D endocrine system and the immune response in rheumatic diseases. abdominal ultrasonography. 86: 327–51. methotrexat and acitretin were used with average improvement.CH50 was observed at T12 in both groups. antybiotics. Italy Introduction: Systemic Lupus Erythematosus (SLE) patients (pts) are at risk for low vitamin D(VitD) levels because of lack of sun exposure. cholelithiasis. During the observation the diabetes.04).A.000UIonce/month). In the most of cases 4 ARA criteria are implemented. Chair and Clinical Department of Internal Diseases.. Conclusions: Intensive supplementation with VitD has a safe profile as the standard but it is able to induce sufficient levels in a larger number of pts. Tansformation to lupus erythrematosus is possible. direct immunofluorescence and allergy tests. diagnosis and co-morbidities A054 A CASE OF PATIENT WITH PSORIASIS. Poland Introduction: Subacute cutaneous lupus erythematosus (SCLE) is a clinically distinct subset of benign cases of lupus erythematosus without organs lesions. Examination of ANA3 profile has shown presence of SSA. I:56%).The other 16pts (group I) were given an ‘‘intensive’’ regimen (Cholecalcipherol 300. The patient has been hospitalizated again in 2012 – the second histopathology and direct immunofluorescence (DIF) were made..The circulating levels of 25OHVitD were dosed every 3months with a chemiluminescence assay(DiaSorin S.6. consisting of skin lesions that are scaly and evolve as polycyclic annular lesions or psoriasiform plaques. Vitam Horm 2011.9 and 12 months ‘‘group I’’ showed significantly higher VitD levels (median at T12:32. At T0 there was no difference in the proportion of sufficient pts (>30 ng/ml) between groups (S:50%. Medical University of Silesia. sensivity to sunlight and accompanying arthralgia have joined. Few prospective studies are available on the effects of VitD supplementation. The HP test stated psoriasis lesions.. but serologically active disease (positive anti-DNA and/or complement consumption in nearly50% of the pts). This process is rare and it is taking many years from the first diagnosis. 864 out of 4. 133 studies were excluded because they did not report outcomes of interest. Materials and methods: We conducted a systematic search using Ovid MEDLINE. Central obesity (2. 2014 .1 1 Division of Rheumatology. A total of 21. there was no relationship between abnormalities in SD-OCT and the dosage of HCQ. 10 (71.4%) had a lower frequency.044 out of 20. United Kingdom ANALYSIS OF SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY (SD-OCT) FINDINGS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS ON HYDROXYCHLOROQUINE THERAPY Ugarte A.130 patients. Fonollosa A. ranging from 10.344 patients were included.8%). The same was true for quantitative thinning of average macular thickness. Two retina specialists analysed qualitatively the scans for abnormalities in the retinal pigment epithelium (RPE) and the outer retina (normal. ‘‘hypertriglyceridaemia’’. Each patient had a complete ophthalmologic examination and the following measurements with SD-OCT (Cirrus. outer retina or both. Methods: Cross-sectional study in a group of 156 consecutive patients with SLE on HCQ therapy from the Lupus-Cruces cohort. Bruce I. 46 studies (58. ganglion cell layer thickness and distribution. UPV/EHU 2Ophtalmology Department.2.5%). MetS prevalence was reported in 23 studies. absolute duration of treatment). Regarding quantitative data. from an initial search of 4. PubMed Central. ‘‘type 2 diabetes’’.607 patients.317 entries were excluded on the basis of titles and abstracts. 19% had qualitative abnormalities of the RPE or outer retina in either eye. Gatto M. Palma L. and hypertriglyceridaemia were the most common components of MetS. ‘‘hypercholesterolemia’’. and impaired fasting glycemia (612/3. or International Diabetes Federation) were considered.1 1 Autoimmune diseases research unit. National Cholesterol Education Program.29% had atrophy of RPE. 45.9th European Lupus Meeting 470 A056 A057 A REVIEW OF METABOLIC SYNDROME IN SYSTEMIC LUPUS ERYTHEMATOSUS: A DANGEROUS OUTBREAK Zen M.2. 16.578.7%) were common manifestations of MetS. ‘‘body mass index’’ (BMI). cross-sectional and cohort studies including SLE patients with measures of MetS. There was no statistically significant difference in the cumulative dose of HCQ between patients with atrophy of RPE/outer retina (474  617. ‘‘Systemic lupus erythematosus’’ and ‘‘metabolic syndrome’’.330 patients. Three established definitions of MetS (WHO.9 g). Restriction to English written papers was applied. and SciVerse Scopus looking for casecontrol. The aim of this study was to evaluate changes in SD-OCT in patients with systemic lupus erythematosus (SLE) taking HCQ and to investigate whether they are related with the cumulative dose or the duration of treatment.com at Institute of Marine Biology of Crete (IMBC) on October 26.6  361. hypertension (8. thickness in each quadrant of the ETDRS grid.938/11.4% to 41. thinned <5%). Diagnostic procedures for MetS should be routinely carried out in SLE patients. University of Padua.X. No difference was found in the rest of the parameters studied (HCQ> 1000g. there was no difference in the frequency of atrophy between patients who had never taken CQ (3 out of 119) and patients who had taken it (2 out of 27). any focal thickening or atrophy). 3. ‘‘waist circumference’’. Lerchundi T. 25.2. duration of treatment. 4. Excluded as not suitable n= 4317 Included after full text review n=74 Reasons for exclusion: Reviews n=54 Not reporting outcomes of interest n=133 Lupus Downloaded from lup. and ‘‘hypertension’’) were used as keywords. Likewise. 54 revision articles were excluded after screening. Hypertension.406/3.1 and Ruiz-Irastorza G.1. obesity.194/4. The University of Manchester.1. The incidence of atrophy of RPE/outer retina was low. We then compared abnormalities in SD-OCT in relation to HCQ cumulative dose.sagepub. Hospital Universitario Cruces. Carl Zeiss): Macular Cube 518x128. Artaraz J. 35. Results: 89% of the patients were female and 95% white.1%) met the diagnostic criteria for MetS.089/4. 12 patients (8%) had thinning of the average macular thickness (<5% of normative data) in either eye. 28. Pernas B. ‘‘hyperglycemia’’. 8 years of treatment. previous intake of chloroquine (CQ) and recognised risk factors for HCQ toxicity (>1000 g cumulative dose and 8 years of treatment). Title and abstract screened n=4578 Potentially suitable n=261 Objective: Spectral domain Optical Coherence Tomography (SD-OCT) is one of the recommended screening tests for hydroxichloroquine (HCQ) related retinal toxicity. thickened >95%. and low HDL cholesterol (1.4%) demonstrated a higher prevalence of MetS in SLE patients compared to controls.4%. whereas hypertriglyceridaemia (1. Conclusions: In this cohort of SLE patients. ‘‘insulin resistance’’. Results: A total of 74 studies were included.366 patients.1.2. Arteagabeitia A.5 g) and those without (507. Articles considering less than 3 components of MetS were excluded. ‘‘HDL cholesterol’’.1. Conclusions: This review presents clear evidence for a high prevalence of MetS in different cohorts of SLE patients. Italy 2Arthritis Research UK Epidemiology Unit. There was no correlation between the average macular thickness and the cumulative dose of HCQ. UPV/EHU Aim: To investigate the prevalence of Metabolic syndrome (Mets) and its components in patients with systemic lupus erythematosus (SLE).3%). or terms linked to each of MetS components (‘‘obesity’’. Borella E. Hospital Universitario Cruces.9%).1. Among 14 case-control studies.2 and Doria A. 39.940 patients. We also recorded quantitative data: average macular thickness and distribution in normative data (normal within 5-95% interval.098 patients (21.722 patients.3%) considered all the components of MetS. HD 5 line raster. high BMI (2. . an aPL.30 [4. Hungary Introduction: Systemic lupus eyrthematosus can bge associated with antiphospholipid syndrome. respectively. and 9 erythrocytes/hpf in urinalysis were associated with type V lupus nephritis. >5 urine leucocytes/hpf.85] for type II. Impaired systolic and diastolic function and increased arterial stiffness have been recognized as surrogate markers of early cardiovascular disease in SLE.8 mm.86 [1. School of Medicine. A059 CLINICAL AND LABORATORY PREDICTORS OF DISTINCT HISTOLOGICAL CLASSES OF LUPUS NEPHRITIS Mavragani C.2. University of Debrecen. with OR [95%CI] for the presence of 3 of the aforementioned risk factors of 4. conventional Doppler and tissue Doppler imaging (TDI) to evaluate systolic and diastolic biventricular function. University of Debrecen have been followed up prospectively for ten years. Greece proteinuria (NRP) were independently associated with III/IV histological classes. Conclusions: The identification of independent factors associated with specific types of lupus nephritis and a risk score for prediction can provide guidance in selecting specific therapeutic modalities. M-mode. Evaluation of larger samples is required to consolidate our conclusions.61[2. University of Athens. Results: 17 females were included. 2014 . Patients and methods: The above mentioned 272 patients with SLE registerered in the Clicical Immunolology Department.P. and mortality in total of 272 lupus patients for ten years of follow up. and Zeher M. Nagy N.09-21. Wave velocity was normal in all patients which may relate to relative short disease duration.3 and Brito I. even in the presence of preserved systolic function. University of Athens.1. E/Vp ratio was abnormal in 3 cases. Portugal. absence of major cardiovascular risk factors and young age sample.1. Aim: To develop risk scores predicting distinct classes of lupus nephritis by using clinical variables and simple laboratory measures. no malar rash. The causes of death were thrombotic events or complications in 40. E/E‘ septal ratio showed increased left atrium pressure in 2 patients. positive anti-dsDNA. School of Medicine. Porto. Age>44 years. median age of 20 years and median disease duration of 8 years. LV systolic function was preserved in all cases. NRP. At baseline. SLE activity.3.3 1 Pediatric Rheumatology Unit 2Pediatric Cardiology Department.76-21] for type V. malar rash. Portugal 3Oporto Faculty of Medicine. an aPLþ group with 81 aPL positive patients without clinical manifestations and a secondary antiphospholipid syndrome (APS) group with 84 aPLþ patients who met the Sapporo criteria. Aim: to assess left (LV) and right (RV) ventricular function and arterial elastic properties in a population with juvenile-SLE.sagepub. RV diastolic function markers were altered in some patients: decreased peak velocity of E wave in 4 patients. Moura C. predicting high mean pulmonary capillary wedge pressure.co-morbidities.2.group with 107 aPL negative patients. Aims: The objective of this study was to investigate of new thrombotic events. A total of 22 new thrombotic complicatons occurred. particularly in cases where renal biopsy is contraindicated. two in the aPL negative group.. 77. Hospital Sa˜o Joa˜o. implying impaired relaxation.com at Institute of Marine Biology of Crete (IMBC) on October 26. A risk score was developed to estimate the risk for developing distinct histological classes of lupus nephritis. Antiphospholipid syndrome seems to negatively influence the survival of the patients with systemic lupus erythematosus. Mitral E/A ratio was normal in 8 patients and showed restrictive pattern in 4. Risk stratification may facilitate therapeutic decisions. Patients and Methods: 163 consecutive patients with biopsy-confirmed lupus nephritis. aortic pulse wave velocity with standard technic (Pulse Trace PWVÕ .2. with AoR: TDI S velocity 9. Results: A total of 28 out of the 272 patients were lost to follow up. The presence of musculoskeletal (MSK) manifestations. negative anti-DNA and 5 urine leucocytes. SLE activity was the most common in the APLþ group but the difference was not significant. Conclusion: Almost all patients had subtle abnormalities of diastolic function..9th European Lupus Meeting 471 A058 CARDIAC FUNCTION AND ARTERIAL STIFFNESS IN JUVENILE SYSTEMIC LUPUS ERYTHEMATOSUS Vieira R. Sza´nto´ A. tricuspid annular plane systolic excursion (TAPSE) 14.7 % in patients with APS. Reduced propagation velocity (Vp) of the mitral valve was observed in 4 cases. 9. Micro Medical) to assess arterial stiffness.2 and Moutsopoulos H. with increased A´ wave velocity in 1 patient. Years ago our study team investigated the thrombotic risk factors and complications in 272 lupus patients with and without antiphospholipid antibodies. Conclusions: Our data confirm recurring thrombotic events in lupus patients with antiphospholipid syndrome. Results: Variables independently associated (p<0. new-onset hypertension. TEN YEARS OF FOLLOW-UP Tarr T. Fragoulis G. RV systolic function was normal in all patients except for one. creatinine levels>1. E/E´ and E/A ratios were normal in all cases. 14 out of 81 aPL positive and 10 out of 107 aPL negative patients died during the follow up. three in the aPLþ group. A060 CLLINICAL OBSERVATION IN LUPUS PATIENTS WITH AND WITHOUHT ANTIPHOSPHOLIPID SYNDROME. Introduction: The type of histological class of lupus nephritis dictates treatment approaches among lupus patients with renal involvement.3 cm/s. when renal biopsy is contraindicated or poses high patient risk. Athens 2Department of Pathophysiology. Gyo00 ri N. Tzioufas A.G.13] for types III/IV and 7. Patients and Methods: Cross-sectional evaluation of a juvenile-SLE patient’s sample: two-dimensional.2. Athens. The majority of patients presented at least one criteria of LV diastolic dysfunction.99-11. Mota C. A total of 27 out of 84 APS (32 %).2mg/dl and absence of nephrotic range Lupus Downloaded from lup.10) with type II class included age  44 years. Department of Clinical Immunology. New antiphospholipid antibodies appeared in these patients.M.2 1 Department of Physiology. absence of MSK complaints or leucopenia.3 % of the new thrombotic complicatins reoccured in the APS group despite the adequat anticoagulant and/or anti-platlet therapy. three groups were created.E.1.. A risk score for the prediction of specific histological classes was calculated for each patient. Introduction: Cardiovascular events are the most common cause of death in systemic lupus erythematosus (SLE). Arterial wave velocity was within normal range in all tested patients (n¼11). Debrecen. The patients undervent physical examination with echocardiography. angina pectoris (13. The SDI was significantly higher in patients who are older then 50 years at the time of diagnosis.Sm antibodies were more often identified in patients with late onset SLE (p¼0.05). Patients and methods: Consecutive SLE-patients (n¼67) were included in a longitudinal follow-up cohort with visits every 2 months for 2 years. as well as in a decrease in serum serotonin concentrations. Forty-nine percent of the patients displayed a HAD anxiety score >8 on at least one occasion during follow-up and the corresponding number for HAD depression score was 42%.37. Results: The number of visits included in the study was 677. Sweden Introduction: Mood disorders are common in SLE. SLE activity was assessed with SLEDAI index. Sza´nto´ A. type 1 interferon activation and serotonin levels may be less influential in determination of SLE-related mood disorder. but anti-cardiolipin antibodies and anti b2GPI were less often identified in patients with late onset SLE than in patients with early onset SLE (p¼0.2.0210).1 and Brzosko M. AND VARY DURING FOLLOW-UP INDEPENDENTLY OF DISEASE ACTIVITY Bengtsson A. and the age at the disease onset influence the disease course. assays for antibodies specific for SLE and for antiphospholipid syndrome were performed. University of Debrecen. 2Department of Psychiatry. Honczarenko K.1 1 Clinic of Rheumatology and Internal Diseases Pomeranian Medical University 2Clinic of Cardiology PUM 3Clinic of Neurology PUM Introduction: Systemic lupus erythematosus (SLE) most often occurs between the age of 20 .81%).035). Aim: The goal of our study was to investigate the chronic organ damages in patient with sytemic lupus registerered in the Clicical Immunolology Department. anxiety and depression scores display a variation over time in SLE in the individual patient. but anemia was far more frequent in patients with late onset SLE (p¼0. Almost half of the patients (46. Debrecen.87 % of our patients. screening and treatment of these complications are important. Lund. Boborowska-Snarska D.05). Ostanek L.1.1.9th European Lupus Meeting 472 A061 COMPARISON OF CLINICAL PRESENTATION AND IMMUNOLOGICAL PROFILE IN SLE PATIENTS WITH THE DISEASE ONSET AFTER THE AGE OF 50 AND IN SLE PATIENTS AGED OVER 50 on´ska D. Variables were dichotomized due to statistical considerations. Conclusions: Despite numerous similarities related to age. Hungary Introduction: The long term survival of patients with systemic lupus erythematosus has progressively improved wordwide. Anti.1 1 Department of Clinical Sciences.013). Lood C. there are differences in clinical presentation and serological profile in SLE older patients and in patients with late onset SLE. Conclusion: Our data confirm that the most common chronic organ damages occur in the cardiovascular or neuro-psychiatric system.sagepub. together with Galectin 3 Binding Protein (G3BP) as a marker of type 1 interferon system activation.1.2. Results: We detected one or more organ damage score in 77. Lund University.043). Higher cummulative steroid dose and using of cyclophosphamide significantly increas the SDI. 2014 . Apart from standard tests. Findings: SLE female-to-male ratio decreases with age. Subjects and methods: 183 SLE patients aged 17-79 were examined.17%). Section of Rheumatology. A large variation could be seen in total HAD score with a mean difference per patient between the maximum and minimum scores during the follow-up time of 10. University of Debrecen.1. Gullstrand B. Patient’s sex. RESULTS OF A HUNGARIAN IMMUNOLOGYICAL CENTRE Gyo00 ri N. The gender did not influence the score of the damage index. Furthermore. A062 DEPRESSED MOOD AND ANXIETY ARE COMMON IN SLE. Conclusions: Clinically significant anxiety and depression is common in SLE during follow-up. The most frequent damages are valvulaopathies (16. Thrombosis was equally common in both groups. neurological examination. The SDI has become significantly higher after the first 10 years existence of lupus.3.A. while hydroxychloroquin treatment is favourable for the patients. Lesions detected by echocardiography and coronary heart disease symptoms were equally frequent in both groups. Hungary.40 years. and additionally electrophysiological examination and imaging tests (MRI and SPECT). Our hypothesis was that a higher disease activity and activation of the type 1 interferon system would be reflected in an increase in depressive symptoms and anxiety. G3BP concentrations or serotonin levels. At every visit patients completed the Hospital Anxiety and Depression scale (HAD) and disease activity was recorded using the SLE disease activity index 2k (SLEDAI-2k). HAD depression and anxiety scores were not associated with SLEDAI scores. Siminszky Zs. Aim: The aim of the study was to compare the clinical presentation and immunological profile in patients with SLE onset after the age of 50 and in SLE patients aged over 50.1 and Tarr T.2 and Jo¨nsen A. Pl Fischer K. but afterwards did not increase anymore. age. Lupus Downloaded from lup.1 1 Department of Clinical Immunology.006).17%) and venous thrombosis (13. It was the lowest (4:1) in patients with SLE diagnosed at the over age of 50 years.3 and a mean standard deviation for the percent change per patient of 0. cognitive dysfunction (14.2.29%). Serositis and polyneuropathy were more frequent in older patients (p<0. For statistical analyses we used Generalized Estimating Equation to handle correlated data. they were more frequent than in younger patients and in patients with SLE diagnosed before the age of 50 years (p<0. Section of Microbiology. Aim: To study anxiety and depression in SLE in a longitudinal setting and to correlate symptoms with disease activity and type 1 interferon activity. Immunology and Glycobiology.1. and they were more frequent than in other patients (p¼0. Serotonin concentration was determined in serum. Disease activity. Lund University.1. Zeher M. The prevention of organ damage become one of the major aims in the management of lupus patients. Sweden 2Department of Laboratory Medicine.com at Institute of Marine Biology of Crete (IMBC) on October 26.50%) had one or two damage(s). University of Debrecen. Csere´p E. Regular follow-up. the highest score was 8. Patients and methods: The authors analyse retrospectively the results of 357 lupus patients with using the internationally accepted SLICC/ACR damage index. Lund. Anti-histone antibodies were more often identified in patients under 50 years of age (p¼0. A063 WITHDRAWN A064 INVESTIGATION OF CHRONIC ORGAN DAMAGE IN PATIENTS WITH SYSTEMIC LUPUS. Pini.5 þ 6 years (51.0%) by focal proliferative glomerulonephritis and 2 (8.005) and lymphopenia (66. Rabat. avascular necrosis of bone.1.8% vs 26. Souissi. Morocco Introduction: the clinical and serological features have been analysed in 70 patients with jSLE(< 18 years).3% and at 15 years 82. survival has improved.9%) resulted affected by diffuse proliferative glomerulonephritis. University of Milan.7% and 38 vs 28% respectively). 5 (17.2. Hydroxychloroquine was administrated in all cases. 18 patients were 12 years old or less. Two patients died of septic shock. followed by cutaneous manifestations in 90 % cases.6%) by membranous nephropathy.4% versus 94. 23 patients had 1 or more renal biopsies: 17 (73. Short stature. which required total hip joint replacement in 1 patient.7% vs 81.1. 3 (13.2% p <0.sagepub. Anti-phospholipid antibodies are present in five patients. Results: there were 3 deaths.. due to disease itself and/or its treatment.. cushingoid features. were an important cause of morbidity. were unfrequent but severe complications in our jSLE series.9%. Results: The female to male ratio is 4. thyroiditis in one case.5%. These patients were compared with a group of 337 patients aged younger than 50 years at SLE diagnosis. therapy and outcome. Objectives: Systemic lupus erythematosus (SLE) is a multisystem inflammatory disease predominantly occurring in females of childbearing age. Conclusions: these data show that jSLE is not necessarily associated with poor prognosis. Out of 28 patients with renal involvement. Prognosis is related to comorbidities and infectious complications. Articular involvement is the most frequent lupus-related symptom present in 95 % cases. Three patients received immunosuppressive drugs. Aim: to evaluate features. Patients and Methods: the mean age of onset was 13 years. One patient has hepatitis C viral cirrhosis and two patients have myelodysplastic syndrom.4%). The most common organ involvement was skeletal: non erosive arthritis. Antinuclear antibodies are present in 96%.5. The mean duration of disease was 9 years. Harmouche H. Becciolini A. Renal involvement was noted in 7 cases (glomerulonephritis class III in 4 cases and class IV in 3 cases). mean age at disease onset is 59.1.78 years). Bachir H. The 5 years overall survival was 90. Lupus Downloaded from lup. followed for one year or more.2 1 Universite´ Mohamed V.1%) all of whom but one recovered with restitutio ad integrum. Method: We conducted a retrospective study to analyze characteristics and outcome of patients with late-onset SLE in a Moroccan tertiary referral center and compare them with those of younger patients with SLE.com at Institute of Marine Biology of Crete (IMBC) on October 26. SLE was associated with Biermer disease in two cases. Infections remain a major problem in morbidity: serious infective manifestations occurred in 15 (21. nephropathy (28.1. 8 cases out of 9 recovered without permanent CNS residue. Department of Rheumatology. anti-DNA antibodies in 71.1 and Adnaoui M.5 vs 46. There was no difference between the two groups for the immunological data.4%).7%). observed in 22 patients (31. Ammouri W. Maamar M. 10 (35. Pediatric Rheumatology Unit. striae rubrae and alopecia were frequent problems. observed in 61 patients (87. Gattinara M.1. Steroids were administrated in eighteen as oral prednisone between 0.8%). particularly in adolescents. that caused increased psychological distress. but morbidity due to disease itself and complications of therapy remain a significant problem.5 and 1 mg/kg per day preceded by a pulse of methylprednisolone in 2 cases. resulting from prolonged corticosteroid treatment in chronically active disease. growth failure and osteoporosis.. Italy LATE ONSET SYSTEMIC LUPUS ERYTHEMATOSUS: RETROSPECTIVE STUDY ABOUT 22 CASES Mezalek Tazi Z. auto-immune hemolytic anemia (19% vs 37. Ibn Sina University Hospital.005). Cutaneous vasculitic lesions. Serositis and thrombopenia occurred more frequently (42. From 1996 to 2012.8%) renal failure. observed in 5 cases (7. Faculte´ de Me´decine et de Pharmacie.2.L. 22 patients were identified as having late onset SLE defined as SLE diagnosed at or over the age of 50 years. CSN involvement occurred in 9 patients (12.8% p<0.5% in early-onset SLE.9th European Lupus Meeting 473 A065 A066 JUVENILE SYSTEMIC LUPUS ERYTHEMATOSUS (JSLE) IN A TERZIARY CENTRE OF PEDIATRIC RHEUMATOLOGY (FEATURES. We compared clinical characteristics. at 10 years 93.7%) showed arterial hypertension. Some manifestations occurred less frequently in late onset SLE .4% and anti-Sm dans 28. Clinical evidence of nephritis (proteinuria) occured in 30 patients (42.8%): 5 seizures and 4 psychosis. The estimated overall survival at 5 years was 97. and Gerloni V. laboratory data. morbidity and morbility of jSLE. 2014 . 3 required dialysis. Rabat.3%. Istituto Ortopedico G. MORBIDITY AND MORBILITY) Pontikaki I.1%).2. Meroni P. This disease is uncommon after the age of 50 years and has different clinical and laboratory characteristics. Maroc 2Internal Medicine departement. Conclusion: The clinical pattern of late onset is characterized by a lower disease severity especially with regard to renal involvement. anti-dsDNAþ (96%). depressive symptoms as BDI > 10.2. Lupus Downloaded from lup.01). Ciapparelli A. 6 (21%) relapsed and 3 (10%) progressed to end stage renal disease.6%.7% vs 22. Neurobiology.2 and Tincani A. but data on long-term outcome are still very limited. Bruno R. Results: In the SLE cohort poor sleep quality was more prevalent than in H (62.9 %. Greece Introduction: Nephrotic syndrome is usually seen in class IV or V lupus nephritis. Conclusion: Lupus podocytopathy is an uncommon cause of nephrotic syndrome in SLE patients.com at Institute of Marine Biology of Crete (IMBC) on October 26.2 male) with diagnosis of PAPS [1] followed from 1984 to 2013 with a mean age of 32 years and a median follow-up of 20.2% vs 22. insomnia as ISI >8.1. p<0. activity. that were successful in 75% of cases. 6. Hippokration General Hospital 3Department of Pathology.1 1 Rheumatology Unit. EM not yet available).1. infective. neoplastic events were recorded in 34%.2. 2014 . and trait anxiety as STAIY2 > 40. Rheumatology and Clinical Immunology. as well as difficulty in maintaining sleep and/or early morning awakening (62.6]). 40%).2% versus 39. A renal biopsy showed in 3/18 glomeruli a ‘‘tip lesion’’ type FSGS and acute tubular injury. A 31-year-old male with history of arthritis (elbows.1.3 % H patients (p<0.1. immunosuppressants p:0. Nineteen patients (57%) had 28 pregnancies. Palagini L.sagepub. Vagnani S.6%. Patients with other sleep disorders were excluded. Unluckily PAPS can be mortal. mildly impaired renal function (Cr¼1.M.07-23.2%. hematuria (46%) and elevated creatinine (43%). hyperlipidemia. Arthritis (73%) and rash (62%) were common. Giannou P.1. low C3/C4 (40%). Conclusions: In a cohort of SLE women. Signorini E.2. A strict control of risk factors and effective treatment are needed to improve the long term outcome. RF.1% of SLE vs 13.001) while anxiety disorders were more common in H patients (H 35.1.5 months. Laboratory work-up revealed positive ANA (1:2. The patient was treated with 3 daily IV methylprednisolone pulses (1 gm) followed by corticosteroids (CS) pos and azathioprine (2 mg/Kg). The prevalence and characteristics of sleep disturbances in Systemic Lupus Erythematosus (SLE) have been rarely studied. A069 POOR SLEEP QUALITY IN SYSTEMIC LUPUS ERYTHEMATOSUS: DOES IT DEPEND ON DEPRESSION? Tani C. a number of cases of SLE patients presenting with nephrotic syndrome and renal biopsy findings consistent with minimal change disease or focal segmental glomerulosclerosis (FSGS) have been reported under the term ‘‘lupus podocytopathy’’. Hemorrhagic.1 1 2nd Department of Medicine and Laboratory 2Renal Unit. Renal biopsy showed podocyte effacement (97%). Pharmacology and Biotechnology 3Hypertension Unit. 14 patients (33%) were treated with CS and another agent (MMF. University of Pisa. were common.001.2.1. Andreoli L.1 and Mosca M. Frassi M.3. References 1 Miyakis S et al. Italy 2Spedali Civili of Brescia.006). Aim: Aim of this study was to describe the outcome of patients with long-standing PAPS. wrists) presented with abrupt onset of lower extremities edema.001). After adjusting for confounders. insomnia and poor sleep quality. Athens Medical School. nephrotic range proteinuria (10 gm/24h). Brescia.9 [Cl 1. Petras D. Organ damage progresses in a significant proportion of patients and it is associated with previous arterial events.5 years (range 15-30) were included.5 [Cl 1. Athens. Patients and methods: Medical records of patients prospectively followed in our center for at least 15 years were retrospectively reviewed.055 and anti-hypertensive p:0. 29/ 43 patients (67%) were treated with CS alone: 20 (69%) showed complete/partial remission. Poor sleep quality was defined as PSQI >5.16]). Deutsch M. arterial 40% and both 10%). Objectives: This study aimed at evaluating the prevalence of insomnia and poor sleep quality and the determinants of sleep quality in a cohort of SLE patients.01). Rheumatology and Clinical Immunology. Ghiadoni L. 51 cases of ‘‘lupus podocytopathy’’ were identified in the English literature over the last 20 years (Medline search through PubMed). p<0.2. mesangial deposits by IF or EM (70%) and glomerular sclerosis (1 glomeruli. serological alterations but for cardiovascular risk factors (less estroprogestinic use p:0. A depressive disorder was observed in 35.500). Fiftythree women with hypertension (without SLE) were enrolled as control group (H). A thrombotic event appeared in 28% of patients during follow up (venous 50%. Conclusions: Despite therapy a high proportion of patients experiences new thrombotic events and severe complications. sleep disorders had a significant association with depression (OR. Recently. A068 OUTCOME OF LONG-STANDING PRIMARY ANTIPHOSPHOLIPID SYNDROME: A SINGLE CENTER EXPERIENCE Taraborelli M. Depressive disorders might be responsible for the higher prevalence of poor sleep quality in SLE. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS).9th European Lupus Meeting 474 A067 LUPUS PODOCYTOPATHY: A CASE REPORT AND LITERATURE REVIEW Thomas K. 87% were female (mean age ¼ 31.002) and therapy (more hydroxicloroquine p<0.2.1. Results: Thirty-five Caucasian patients (33 female. Koutsianas C.1. Reggia R. Italy Introduction: Acute manifestations related to Primary Antiphospholipid Syndrome (PAPS) are well known. Querci F.Department of Clinical and Experimental Medicine. One patient with chronic bowel ischemia died for sepsis. Franceschini F. Patients and Methods: Eighty-one consecutive SLE female patients were evaluated in a cross-sectional study. hypoalbuminemia (1. p<0. CYC. Gemignani A.6 years) with a median disease duration of 2. Carli L. 2Department of Psychiatry. especially difficulty in maintaining sleep. Dall’Ara F. Brescia. p¼ns).027) in particular arterial (p<0. SLE was associated with higher risk of having poor sleep quality (OR.6 mg/dL). Kidney biopsy is essential for diagnosis while steroids alone can induce remission in the majority of patients. while insomnia was similarly prevalent (29.001).1 1 Spedali Civili and University of Brescia.J Thromb Haemost 20064(2):295-306. the Beck Depression Inventory (BDI) and the STAI-Y2 were administered.3. Bombardieri S. damage.3. We report a case reviewing all cases reported in the English literature. Italy Introduction: Sleep disturbances are frequently observed in patients with rheumatic diseases and significantly affect their quality of life.4.1. The Pittsburgh Sleep Quality Index (PSQI). Pregnancy outcome is quite good. while no correlations were observed with disease duration. Laboratory findings included: ANAþ (88%).2. Case description. CsA) with 11 showing complete or partial remission. Gakiopoulou H.8% vs SLE 16. 2. 6% and 9% respectively.2 and Vassilopoulos D.5 mg/dL) and low C4. anti-RNP and anti-Ro antibodies. the Insomnia Severity Index (ISI). No significant variations were observed between the onset and follow-up for systemic involvement. Department of Clinical and Experimental Medicine. IF was positive only for IgM (1/4 glomeruli.1.21-5. Organ damage was present in 20% of patients (17% neurological and 3% renal) and was significantly associated to the occurrence of thrombotic events (p:0. Taglietti M. 0 95. p<0. Conclusions: In this large Portuguese lupus cohort.J.556 0.com at Institute of Marine Biology of Crete (IMBC) on October 26.hematologic (%) SLICC-DI 1 (%) 41. Mazur M.6.1. Nero P5.2 3. mean disease duration .7.000 0.1 and Bartos D.25 (1. The median duration of SACQ-SLE period was 140 [98-294] days.7 14.4 43.1.pt/LES.6. Using the stricter definition (Group 1) SACQ-SLE was found in only 23 patients. Davila’’.1. Results: Of the 640 patients with 2 visits registered in Reuma.3. 48: 1506–1511. Introduction: The majority of patients with systemic lupus erythematosus (SLE) is clinically and serologically concordant. Methods: A prospective study of 76 patients. Mazur-Nicorici L. Seven patients (2. References 1 Prabu A. Testemitanu’’.001) but no correlation with SLEDAI (r¼0. 2University of Medicine ‘‘C. The prevalence of auto-antibodies (anti-Sm.55 (5.9th European Lupus Meeting 475 A070 A071 PULMONARY ARTERIAL HYPERTENSION IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS IN MOLDOVA LUPUS STUDY Cebanu M.43.8 68.PT/LES Santos M.2 ( 6. Conclusion: The incidence of PAH in our study was 2. 2012) classification criteria for SLE.1.neuropsychiatric (%) .7% and the majority were with moderate severity. Romania SEROLOGICALLY ACTIVE CLINICALLY QUIESCENT SYSTEMIC LUPUS: RESULTS FROM THE PORTUGUESE REGISTRY REUMA. that were recruited from rheumatologic department between November 2012 and December 2013. Rheumatology Oxford Journals 2009. Oliveira M.236 27.860 Lupus Downloaded from lup. da Silva J. Roma˜o V.13. Gonc¸alves M.1 96. The implications of isolated serological activity are not fully understood and there is no consensus on the best therapeutic approach for this particular group of patients.8) points.sagepub. PAH was moderately associated with duration of disease r¼0. but in some cases.pt/LES and SLEDAI-2K of 2 or 4 due to hypocomplementemia and/or positive anti-dsDNA antibody titers in at least 2 consecutive visits at >3 months interval were included.4 and Canha˜o H.000 1.4.renal (%) .009. Instituto de Medicina Molecular 3Hospital de Santa Maria.01.159 0. antiRNP or aPL) is similar between groups. Lisboa 6Centro Hospitalar Cova da Beira. Covilha˜ 7Hospital Conde de Bertiandos.05. Patients and methods: Patients with the diagnosis of SLE followed in the Portuguese registry Reuma.J. p<0. The data of incidence of PAH in SLE are controversy.7%) were found to have PAH with sPAP>30mmHg.3. Prevalence and risk factors for pulmonary arterial hypertension in patients with lupus. Sadovici V.9 76.2 1 State University of Medicine and Pharmacy ‘‘N. 2014 .2.6%) fulfilled the inclusion criteria. Ponte de Lima.7 12.1.61.2..7 11.2 21 30. Ineˆs L.65). 55 (8. mean SLICC criteria number – 6. 2 patients had 40 – 45mmHg and there was just one patient with 70mmHg. Characteristics Group 1 (n¼23) Group 2 (n¼32) p-value Age (years) Female (%) Caucasian (%) Disease duration (years) Previous involvement: . The activity of SLE by SLEDAI was 12. There was a significant correlation between PAH and SLAM (r¼0. PAH was defined as systolic pulmonary artery pressure (sPAP)>30mmHg. among them 4 patients had 32 – 40mmHg. Results: From 76 patients who entered in the study 73 were female (92.7. SACQ is uncommon.1. The activity of disease was estimated by Systemic Lupus Disease Activity Index (SLEDAI) and Systemic Lupus Activity Measure (SLAM). Republic of Moldova. Evaluation of pulmonary artery pressure and cardiac morphology was performed at rest with Transthoracic Doppler Echocardiography as a screening tool.1%) with median age 44 (12) years. range 1-360 months.8 1. anti-Ro. Table 1. Coimbra 5Hospital Egas Moniz. Lisboa 4 Hospitais da Universidade de Coimbra. Portugal Introduction: Pulmonary arterial hypertension (PAH) is a serious complication of systemic lupus erythematosus (SLE) which is associated with a significant risk of death [1].7 0. Objectives: To estimate the incidence of PAH in SLE patients and to identify the correlation with disease activity. Pasali M. Aim: To identify the frequency and characterize patients with serologically active clinically quiescent (SACQ) SLE.P. Sousa S.A.62.9) years. Almada 2Rheumatology Research Unit. 23 (42%) are treated with antimalarials or no medication (Group 1) while the remaining 32 are medicated with corticosteroids and/or immunosuppressive drugs (Group 2). Cerqueira M. anti-La.76 (8.3 1 Hospital Garcia de Orta.2.9) and by SLAM .3 4.565 0.4. who fulfilled Systemic Lupus International Collaborating Clinics (SLICC.9572) was observed. et al. No clinical or immunological differences could be identified between SACQ patients receiving corticosteroids and/or immunosuppressants and those receiving only antimalarials.3 56. high titers of anti-dsDNA antibodies and/or hypocomplementemia persist in the absence of clinical manifestations. range 4-10. A significant correlation was observed between PAH and SLAM that may confirm its sensibility versus SLEDAI.2.298 0. p¼ 0.3 87. metacarpophalangeal and metatarsophalangeal joints. Greece 2General Hospital of Athens ‘‘G.9th European Lupus Meeting 476 A072 A073 SYSTEMIC LUPUS ERYTHEMATOSUS PRESENTING AS SEVERE RELAPSING NEUROMYELITIS OPTICA Avdikou M. Intensive Care Unit. 2014 . 1General Hospital of Athens ‘‘G.1.1. Markoulaki D. Rabat. Brain and spine MRI scan reported multiple hyperintense (in T2-FLAIR weighted imaging) non-enhancing lesions in the medulla. Zdrenghea D. Greece THE IMPAIRMENT OF LEFT VENTRICULAR SYSTOLIC PERFORMANCE IN SYSTEMIC LUPUS ERYTHEMATOSUS Pamfil C.1. 81% female) and 20 matched healthy controls underwent transthoracic echocardiography (chamber diameters.2. . Cluj.Gennimatas’’. Blood test results revealed leucopenia. the majority categorized as mild. Moreover hyperintense lesions were detected in T2-STIR weighted images from the medulla to T7 level with mildly enhancing lesions at the level of T5. Cerebrospinal fluid analysis showed pleocytosis (>600cells/mm3. The purpose of this study is to analyze the clinical and biological features. Maroc 2Internal Medicine departement. Karageorgas T.038). Harmouche H. Ammouri W.2*. Case Description: A 57 year-old female patient on azathioprine and prednisolone for recurrent episodes (n¼ 6) of NMO (aquaporin-4 antibody positive) presented with dry cough. Rheumatology Department. Aim: To assess the presence of myocardial systolic impairment in patients with SLE by 2D echography and speckle-tracking strain analysis and to investigate its relationship to immunological and clinical features in SLE patients. Conclusion: Patients diagnosed with NMO should be screened by a rheumatologist for an underlying systemic autoimmune disease (SLE or Sjogren’s syndrome) as neurologists may miss subtle evidence of these diseases. anti-dsDNA positive and low levels of C3 and C4.45  1. T6 and T7 (Figure) consistent with acute myelitis.21 % vs. Romania Introduction: Neuromyelitis optica (NMO) has been associated with Systemic Lupus Erythematosus (SLE) occasionally representing the initial manifestation of the disease.1. Ibn Sina University Hospital. using ACR criteria. evolution and related factors of neuropsychiatric complication of SLE. which damaged may contribute to impaired functional capacity of patients with SLE. LVEF. 20.6 0. malar rash. clinical data.1.1. The average age was 3011 years. Conclusion: There is a significant impairment of the systolic myocardial performance. Patients – Methods: It’s a retrospective monocentric study of 348 cases diagnosed between 1990 and 2010 in an internal medicine department. Results: Out of the 38 patients. Furthermore.15  1.1 1 Iuliu Hatieganu University.1 and Rednic S.1 *Equal contribution. GLS values were lower in patients with associated antiphospholipid syndrome (APS) (18. Kalogeromitros A.045).3 and Papakonstantinou K. Lintzeri A. The central Lupus Downloaded from lup.5  1. Maamar M.49 %. protein of 990mg/dL and normal glucose concentration.1*. disease activity and damage scores were additionally obtained.1. disease duration did not correlate with GLS. p¼0. Patients and Methods: Thirty-eight asymptomatic SLE patients (mean age 44  5. Ultrasonography allows accurate evaluation of the myocardium. Athens. pons.2 1 Universite´ Mohamed V. 31% had mild mitral regurgitation and one patient (2%) had mild aortic regurgitation. Pop D. Medical School. corona radiata and centrum semiovale. Standard 2D measurements were similar in both groups.1. Morocco Introduction:The neuropsychiatric manifestations are one of the the most deadful manifestations of systemic lupus erythematosus (SLE). Flestea A. Faculte´ de Me´decine et de Pharmacie.3%) mostly in women (95%).1. A074 THE NEUROPSYCHIATRIC MANIFESTATIONS OF SYSTEMIC LUPUS . In LES patients.Gennimatas’’.2.1. Mardale M. Introduction: Cardiovascular disease is increasingly acknowledged as a main cause of mortality and morbidity in systemic lupus erythematosus (SLE).2. The neurological disorders were inaugural in 85%. Results: The neuropsychiatric manifestations were observed in 95 cases (27. Athens. dyspnea and fever (39oC) and progressively deteriorating weakness of all four limbs over the past month.1. No correlation between the presence of standard cardiovascular risk factors and GLS values in patients with SLE was observed. Greece 3National and Kapodistrian University of Athens. On examination she had dyspnea at rest and flaccid paralysis with absent reflexes of the upper limbs and decreased muscle strength (2/5) with hypertonia and brisk reflexes of the lower limbs. Rabat. These findings support the usefulness of an early and accurate ultrasound assessment of the heart in order to improve the morbidity and mortality among SLE patients.A MONOCENTRIC STUDY OF 95 CASES Tazi Mezalek Z. Nousia O.1.sagepub. No significant correlations between age and GLS were observed in neither groups. Souissi. erythematous rash of the neck and upper back were also detected. valve abnormalities and LV systolic performance by speckle tracking imaging). midbrain. Chest CT scan showed bilateral lower lobe infiltrates with pleural and pericardial effusion. Increased values of pulmonary systolic pulmonary arterial pressure suggestive for pulmonary hypertension were recorded in 23% in SLE patients. p¼0. Boumpas D. Mechanical ventilation was required due to acute respiratory failure. Synovitis of the wrist. 70% neutrophils).03 % vs 19. She received iv methylprednisolone and IVIg for five consecutive days with resolution of the pulmonary infiltrates and serositis and stabilization of the neurologic disease to be followed by Rituximab. Athens. cerebellum.2. Bachir H.1.2 and Adnaoui M. Romania 2Babes-Bolyai University. but which correlates with APS and damage accrual.com at Institute of Marine Biology of Crete (IMBC) on October 26.T. which doesn’t result from the presence of known CV risk factors. Gusetu G. ANA>1/ 1280. but global longitudinal strain (GLS) was significantly reduced in the SLE group (19. Cluj.9 years. A complete work up for infections yielded negative results.63 %. 2 Gabba A. and reveals structural damage in a large number of patients. aseptic meningitidis in 7 (7.1 and Rednic S.51:2278-2285.patients and directly correlated with both proteinuria (r¼0. The antinuclear antibody were positive in 93 (97.com at Institute of Marine Biology of Crete (IMBC) on October 26. Ghirardello A.1%).024.5%) patients deceaded mostly by infectious complications (P< 0. the DNA antibody in 78 (82. Lupus Downloaded from lup. US detected additional 2/3 grade synovitis in 5 patients.343.0001). Italy. cranial nerve involvement in 5 (5.4%). Thus.001). and to test whether antiPTX3 antibodies are protective against LN in murine lupus.1. Results: Upon clinical examination.M.1. few studies evaluated joint and tendon involvement in SLE patients by ultrasonography (US) and the incidence and the nature of US abnormalities differ among studies.2%). vascularitis and myelopathy in 5 patients each (5. Ta˘mas¸ M. ischemic stroke in 6 (6. References 1 Delle Sedie A.023). p¼0.I. a negative association of anti-PTX3 with LN was found (R2¼0. Prevalence of LN was higher in anti-C1qþ/ anti-PTX3.3%). proteinuria-free survival (300mg/dl) and overall survival were compared. Group 1 mice developed anti-C1q and anti-DNA antibodies later and at significantly lower titers than group 2 (p¼0. Pamfil C. 39% both) with a 2/3 grade in 23% of the cohort.1%) and the anticardiolipin antibody in 19 (20%).1.05) and an anti phospholipid syndrome (P¼0. Aim: To assess the link between serum anti-PTX3 and anti-C1q antibodies.2%) and acute polyradiculoneuropathy in 4 (4. 10 NZB/NZW mice were injected with 100 mg PTX3 in 100 ml PBS (group 1) and 10 NZB/NZW mice with PBS 200 ml (group 2). Padua.1. All patients with clinical arthritis had grade 3 synovitis(3/35). psychotic troubles in 23 (24. anti-PTX3 and anti-DNA antibody titers. Bone erosions were described in 29%.2%). Clin Exp Rheumatol 2009.27:897-901. Zen M.than in C1qþ/anti-PTX3þ or C1q-/anti-PTX3.49. PD abnormalities were present in 14% of patients. et al.1 and Doria A1 Division of Rheumatology. Clinical examination and US evaluation (Gray Scale and Power Doppler (PD)) of the wrist and hand was performed bilaterally.001).1%).001).2 1 Emergency County Clinical Hospital. p¼0. Iaccarino L. and 9% arthritis. 20% of SLE subjects had arthralgia. Group 1 mice displayed significantly longer proteinuria-free survival and overall survival (p¼0. Aim: To describe and determine the frequency of US abnormalities of hand and wrist in SLE. Bassi N. Conclusion: In our report.3%) patients.47.0001). Rheumatology 2012. neurological manifestations were frequent and associated with poor prognosis. Bettio S.008 and p¼0. thrombocytopenia (p¼0. All patients were treated with corticosteroids. Among healthy controls.and no LN was observed in anti-C1q-/anti-PTX3þ patients (p<0. histopathology and imaging A076 ANTI-C1Q-ANTI-PTX3 ANTIBODY BALANCE INFLUENCES NEPHRITIS PROGRESSION IN HUMAN AND MURINE LUPUS Gatto M. 2014 . PTX3 deposition was evaluated on 29 biopsies by immunohistochemical analysis and kidney fibrosis by Masson’s staining. 20 age-matched healthy subjects were included as controls. Rıˆnzis¸ M.0001).1.002). Anti-C1q antibodies are more prevalent and anti-PTX3 less prevalent among SLE patients developing lupus nephritis (LN). notably. renal PTX3 and LN in SLE patients. Tendon involvement is frequent and shows a specific pattern: the involvement of wrist extensors.1. respectively). The peripheral neurological manifestations (17%) were peripheral neuropathy in 12 patients (12. but only 60% of them had synovitis at time of examination. Materials and Methods: Levels of anti-PTX3 and anti-C1q antibodies were measured on sera of 130 consecutive SLE patients by home-made ELISA.024) and kidney fibrosis (r¼0.2%) and thrombophlebitis in 2 (2. Patients and methods: 35 randomly selected SLE patients were enrolled in the study. 13% MCP/PIP. without any accompanying PD abnormalities or structural damage. US examination revealed abnormalities in 77% of cases. involving the wrist extensors in the vast majority of cases (81%). leukopenia (p¼0.6%).1.1%). Conclusions: The presence/absence of anti-C1q and anti-PTX3 antibodies may influence the progression of LN in humans. Department of Rheumatology 2 ‘‘Iuliu Hat¸ieganu’’ University of Medicine and Pharmacy. Conclusions: US abnormalities are common in SLE patients. University of Padova.7%) with immunosupressive agents.0001). US examination detects significant synovitis of the hand and wrist in the absence of clinical findings. Results: Anti-C1q levels were significantly higher and anti-PTX3 significantly lower in patients with LN than non-renal patients (p<0.2. US identified tenosynovitis in half of patients. Two-tailed t test. Anti-PTX3 antibodies may hinder LN onset in murine lupus. Fisher’s exact test and Pearson’s correlation test were performed. Background: Soluble long pentraxin 3 (PTX3) is an acute phase protein involved in clearance of complement fragment 1 (C1q). 98% with antimalarial and 52 (54. Cluj-Napoca. et al. Anti-C1q.1. US may help aid monitoring of joint disease activity and modulate treatment strategies in SLE patients. p<0. located at the site of the second and fifth MC heads in two thirds of cases.8%).sagepub. Nalotto L. US abnormalities were detected in 20% of cases. Anti-PTX3 antibodies appeared only in mice injected with PTX3 (p<0.2. The factors significantly associated with neurological events were: a high SLEDAI score (P< 0. headaches in 40 (42. Biomarkers.028).1.1. Renal PTX3 deposition was highest in anti-C1qþ/anti-PTX3.9th European Lupus Meeting 477 neurological manifestations (83%) were: convulsions in 24 patients (25. The most common finding was synovitis in 66% (48% wrist. The evolution was good in 51 patients (53%) but a relapse was observed in 34 (35. Ten (10. mice survival rates were evaluated by Kaplan-Meyer method using Mantel-Cox for comparison. 3 times 3 weeks apart. A075 ULTRASOUND EVALUATION OF WRIST AND HAND IN SYSTEMIC LUPUS ERYTHEMATOSUS Popov H. Romania Introduction: To date. Cluster 2: anti-dsDNA. 2 Dougados M. Italy Introduction: Childhood-onset systemic lupus erythematosus (cSLE) represents a more severe phenotype of SLE than adult-onset disease. Online J. Pilkington C. Department of Pediatrics. antichromatin. Department of Paediatric Rheumatology. Liphaus BL.pubmedcentral. Kamphuis S. protein markers (neutrophil gelatinaseassociated lipocalin). anti-chromatin/ nucleosome. and early diagnosis and recognition of complications are especially important. Genova. anti-Sm anti-dsDNA Anti-dsDNA is highly specific for SLE Distinct serologic and clinical correlations with autoantibody clusters are shown in South Chinese cSLE Levels of C1q and C1qAb correlate with severity of cSLE and could be of use in diagnosis of nephritis anti-NCS Ab had high cSLE sensitivity and specificity. Campos LMA. Utrecht.ncbi.com/search/ results?subaction¼viewrecord&from¼export&id¼L351288608. and Clinical Immunology. Liverpool. [Internet]. Available from: http:// www.gov/articlerender. fcgi?artid¼3583675&tool¼pmcentrez&rendertype¼abstract. Paris. Ravelli A. UK 8Gaslini Children’s Hospital. Specific anti-nuclear antibodies such as anti-NCS. antiLa.nih. and implementation of recommendations endorsed by the EULAR standing committees. anti-dsDNA. Lupus [Internet]. Department of Pediatric Immunology and Rheumatology. Singer NG. London. 3 Wulffraat NM.8. EULAR guidelines for recommendations were used to assess study validity and level of evidence.3.15(8):496–500. Rotterdam. Rheumatol. Available from: http://www. Kone´Paut I. Rosseto EA. Olson J. Mangueira CLP.S. Bras. Department of Pediatric Immunology.6. anti-Ro. Anti-C1q-antibodies are rarely found but are of use in cSLE diagnosis in anti-dsDNA-negative patients.1. France 6CHU de Biceˆtre.gov/pubmed/16942001. Department of General Pediatrics and Pediatric Rheumatology. antiribosomal P. Scheinberg MA. Mangueira CLP. Y. et al.nih. Ann.nlm. Rheum. antiribosomal P) and complement factors (anti-C1q). Pediatr. Aim: A systematic literature review was performed to analyse the usefulness of biomarkers for diagnosis and monitoring of cSLE.60(9):2772–81. 7 Jesus AA de. Child Health (Oxford).63(9):1172–6. Carneiro-Sampaio M. Reumatol. Levels correlated with SLEDAI score Lupus Downloaded from lup. Neutrophil gelatinase-associated lipocalin is a predictor of the course of global and renal childhood-onset systemic lupus erythematosus disease activity. Suzuki M. Ann. Paediatr. Burmester GR. [Internet]. 2006 Jan [cited 2013 Aug 28]. Euller-Ziegler L. Pediatric Rheumatology. Dis. et al.pubmedcentral. Klein-Gitelman M.nih. France 7Great Ormond Street NHS Hospital. Antinucleosome antibodies in patients with juvenile systemic lupus erythematosus..sagepub.com at Institute of Marine Biology of Crete (IMBC) on October 26. Slovenia 5Necker Hospital.3 1 Wilhemina Children’s Hospital/UMCU. Department of Pediatric Rheumatology. Available from: http://www. Sheinberg M. Author (et al) Validity Level of Evidence Biomarker studied Campos L 2006 (4) Hinze C 2009 (5) Valid Valid 2A 2A Jesus AA 2009 (6) Jesus AA 2012 (7) Jurencak R 2009 (8) Moderate Moderate Valid 2B 2B 2A Antinucleosome antibodies Neutrophil Gelatinase-associated Lipocalin (NGAL) Anti-C1q Antibodies Anti-C1q Antibodies Spectrum of autoantibodies Lehman TJ 1980 (9) Tang X 2010 (10) Moderate Valid 2B 2B anti-dsDNA Spectrum of autoantibodies Wu F 2011 (11) Moderate 2B Anti-C1q and Anti-C1q Antibodies Wu JF 2006 (12) Valid 2B Antinucleosome (anti-NCS) antibodies Results Correlates with malar rash.J. Tekano J.nih. Sci. anti-RNP.fcgi?artid¼1755117 &tool¼pmcentrez&rendertype¼abstract. [Internet]. SLEDAI-score NGAL levels may be of use in predicting global and renal cSLE disease activity Despite low occurrence. Bader-Meunier B. Silva CA. Mangueira CL.2. Malleson. Time to share.1173:235– 8. Department of Women’s & Children’s Health.18(2):61–9. anti-RNP. Anti-C1q antibodies in juvenileonset systemic lupus erythematosus. Department of Allergology. Acad.gov/ articlerender. antichromatin. Diagnosis and management of systemic lupus erythematosus in children. [Internet]. and anti-dsDNA antibodies in juvenile systemic lupus erythematosus patients. Results: After the initial search. Anti-C1q. Division of Pediatric Rheumatology. 5 Hinze CH. 2006/09/01 ed. Available from: http://www.1. et al. Carneiro-Sampaio M. [Internet]. 2012/12/12 Table 1. EULAR standardised operating procedures for the elaboration. Conclusion: Systematic review of literature identified biomarkers that could aid in diagnosis and monitoring cSLE disease progression. anti-ENA. Biomarkers can help significantly in achieving this. anti-C1q Ab is useful in cSLE diagnosis if anti-dsDNA is negative Antibody clusters correlate with different disease manifestations: Cluster 1: anti-dsDNA. 2013 Jan [cited 2014 Jan 13].W. Rev. anti-ENA). Available from: http://www. 2009/08/29 ed. Marie SK. Table 1 summarizes the results of the identified studies.9th European Lupus Meeting 478 A077 BIOMARKERS IN CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS: A SYSTEMATIC REVIEW Groot N. 6 Jesus AA. EMBASE and Cochrane. Rheumatology Service. The Netherlands 2Sophia Children’s Hospital/EMC. University of British Columbia. the Netherlands 3 Alder Hey Children’s NHS FoundationTrust Hospital.gov/pubmed/19758156. [Internet]. anti-Ro. N. Guillemin F. Vastert B. Silva CA. 2009/09/18 ed. Passo MH. Ljubljana. 83 articles on biomarkers in cSLE were identified. 2004 Sep [cited 2014 Jan 13].M.nih. The following biomarkers were identified: anti-nuclear antibodies (antiNCS. P. anti-La. Vastert S. Rheumatology. 2008.embase. 2009 Sep [cited 2013 Aug 28]. Paris. anti-ribosomal antibodies (anti-U1RNP. dissemination. anti-Sm. Hirvonen J. Most studies combined paediatric and adult data.4. References 1 Malleson P. evaluation.gov/ articlerender. A minimum validity score of ‘‘moderate’’ and level of evidence of 2B (case controlled studies) was set for inclusion. and NGAL levels are promising biomarkers for monitoring disease activity. anti-U1RNP. a good marker for cSLE diagnosis. Methods: The search was executed in MEDLINE.ncbi. Avcin T.5. 4 Campos LMA. 2014 . This study was part of the SHARE Project (Single Hub and Access point for paediatric Rheumatology in Europe).7.11(1):5. Cluster 3: anti-dsDNA. Betteridge N. anti-Sm. Canada. Arthritis Rheum. et al. 2009 Sep [cited 2013 Aug 27]. Available from: http:// www. Kiss MHB.2 and Beresford M. United Kingdom 4University Medical Center.fcgi?artid¼3064260 &tool¼pmcentrez&rendertype¼abstract. haemolytic anemia.pubmedcentral. Nine articles regarded pediatric data only and were eligible for analysis. Vancouver.nlm. Medicine (Baltimore). The DSCI-MRI technique was applied and regional cerebral blood volume (CBV). [Internet].3.001). Singsen BH.0025. Stockholm. APRIL. 2010 Jan [cited 2013 Aug 28]. Results: NPSLE patients had lower CBF values and higher MTT values than HV. as compared to SLE-controls. C1q and anti-C1q antibody levels are correlated with disease severity in Chinese pediatric systemic lupus erythematosus. at either baseline or follow-up. J. and mean transit time (MTT) values were estimated in 20 regions of interest (ROIs). Among PLN patients.ncbi. Serum samples at baseline and follow-up were analyzed for BLyS. Fanouriakis A.gov/pubmed/6965717.gov/pubmed/23223707. 1980 Apr [cited 2013 Aug 28]. Zhao Q. Tyrrell P.nih.001) and anti-C1q (p<0.1 1 Department of Medicine. Bernstein B.32:283–288. PLN.control patients and HV.ncbi. After induction therapy a follow-up biopsy was performed. Lupus Downloaded from lup. Stockholm University. the decrease of APRIL was significant only in responders (p¼0. Results: Comparing patients to controls. No correlation was found between BLyS/APRIL and anti-dsDNA. 12 membranous LN) and 64 individually matched controls were included. Int.gov/pubmed/ 19208567. Cui XD. and MTT values between SLEcontrols and HV.1. 10 Tang X. Sidiropoulos P.nlm. and clinical correlations. Clinical response (CR) was defined as 50% reduction in proteinuria. NPSLE patients had lower CBF values in DGM and higher MTT values in NAWM and DGM. but have limited anatomic resolution and high-radiation dose. Lower CBF values in NPSLE patients. 2011 Apr [cited 2013 Aug 28]. Hanson V.ncbi. [Internet]. Eur J Nucl Med 1997.BLYS AS A NON-INVASIVE PREDICTOR OF RESPONSE Parodis I. 2006/08/22 ed. Available from: http://www. Stockholm. Clinical and serologic correlations and autoantibody clusters in systemic lupus erythematosus: a retrospective review of 917 patients in South China. Through comparison with clinical data and correlation with autoantibodies.sagepub. 12 Wu J-F. Autoimmun.ncbi. There was no significant difference in CBF. and among PLN patients (rs¼0.gov/pubmed/ 16919912. Zhang X. Rheumatol. cluster analysis. 1980/04/01 ed. placed in NAWM and DGM structures bilaterally. Benseler S. 2009/12/25 ed. 9 Lehman TJ. Tang L. Available from: http:// www. cerebral blood flow (CBF). 24:787–91. CBV. Shen E-Y. across all 20 ROIs.1. Axelsson M. 2014 .5 ng/mL) predicted treatment response. Silverman E. Antinucleosome antibodies correlate with the disease severity in children with systemic lupus erythematosus.2.control patients and HV. Compared to SLE-controls. 2Rheumatology and 3Medical Physics. and hypoperfusion in NAWM compared to HV. Svenungsson E. Low baseline BLyS levels (<1. Sweden Introduction: Lupus nephritis (LN) affects up to 50% of patients with Systemic Lupus Erythematosus (SLE). University Hospital of Heraklion.1 Departments of 1Radiology.005). anti-C1q. Bertsias G.nlm.gov/pubmed/20075706. p<0. Conclusions: NPSLE patients are characterized by diffuse significant hypoperfusion in DGM compared to SLE. However.nih. and stress the need to evaluate BLyS as a candidate non-invasive prognostic biomarker of treatment response in LN. Lee J-H.nlm. [Internet]. Zickert A.1. 2009 Feb [cited 2013 Aug 28]. The B-lymphocyte is pivotal in SLE and autoantibody production. attaining a positive predictive value of 92% for CR among PLN patients. Maris T.52(6):976–81.2 and Karantanas A. A078 BLYS AND APRIL IN LUPUS NEPHRITIS: CORRELATIONS WITH SEROLOGY .T. Available from: http://www. Sweden 2AlbaNova.ncbi. but higher CBV values only in NAWM.89(1):62–7. 11 Wu FQ. Greece Introduction: PET and SPECT studies revealed hypometabolism and hypoperfusion in NAWM and DGM regions in NPSLE patients (1.009 for CR).com at Institute of Marine Biology of Crete (IMBC) on October 26.001) and remained unchanged after treatment. Pediatr. Group differences on perfusion values were assessed using one-way ANOVAs. Huang Y. J. Rheumatology Unit. Wang L-C. p<0. [Internet].96(4):657–61.27(2):119– 24. Aim: To investigate possible hemodynamic impairment in NPSE patients. 2). B-lymphocyte stimulator (BLyS) and a proliferation-inducing ligand (APRIL) are important for the activation and maintenance of B-cells. There is no significant hemodynamic differences between SEL. Kavroulakis E. References 1 Appenzeller S et al. 46:467–472.05/20 (ROIs) ¼ 0.9th European Lupus Meeting 479 ed.2. Rheumatol. The role of antibodies directed against double-stranded DNA in the manifestations of systemic lupus erythematosus in childhood. Aim: We investigated serum levels of BLyS and APRIL in patients with LN to clarify how these levels are affected by conventional immunosuppression.47. [Internet].1 and Gunnarsson I.49.gov/pubmed/20033414. Boumpas D. separately for each ROI and evaluated at Bonferroni-adjusted a ¼ 0.nih. Rheumatology 2007. we further aimed to evaluate BLyS and APRIL as potential biomarkers for LN in comparison to conventional serology. APRIL levels were significantly higher in patients at baseline (p¼0.2. normal or improved renal function and inactive urinary sediment.31(4):501–5. Available from: http://www. 2012 Dec [cited 2013 Aug 28]. Weng W. significant correlations were found between BLyS and anti-dsDNA in the combined patient group (rs¼0. 2006 Sep [cited 2013 Aug 28]. Patients and Methods: Sixty-four patients with active biopsy-ascertained LN (52 proliferative LN. but its application in NPSLE is still very limited (3).36(2):416– 21. Autoantibodies in pediatric systemic lupus erythematosus: ethnic grouping. Available from: http://www. 2 Otte A et al. J Magn Reson Imaging 2010. Available from: http://www.nih. Significant decreases of anti-dsDNA (p<0.1. autoantibodies and complement levels. King K. 2010/01/16 ed. baseline BLyS levels were significantly higher in patients (p<0. using DSC-MRI. 20 SLE patients without CNS involvement (SLE-controls) and 25 healthy volunteers (HV) were administrated brain MRI studies. reached significance in periventricular NAWM and semioval center. Karolinska Institutet. but not at follow-up. Kornreich HK. Deng W. 2009/02/12 ed.nlm.nlm.1. A079 DYNAMIC SUSCEPTIBILITY CONTRAST MAGNETIC PERFUSION IMAGING (DSC-MRI) TECHNIQUE IN NEUROPSYCHIATRIC SYSTEMIC LUPUS ERYTHEMATOSUS (NPSLE): DIFFUSE HYPOPERFUSION IN NORMAL APPEARING WHITE MATTER (NAWM) AND DEEP GRAY MATTER (DGM) Papadaki E.nih. C3 or C4. 3 Emmer BJ et al. Yang Y-H. Zhang W. 8 Jurenca´k R.nih. Fritzler M.001). Conclusions: Our results indicate that APRIL is of importance in PLN. J.001) were observed in PLN patients. Patients and Methods: 25 NPSEL patients. Hiraki L.nlm.ncbi. Chiang B-L.2. Malmstro¨m V. The DSC-MRI technique is widely used for the detection of hemodynamic changes in CNS diseases. 3 4.1 2. matrix metalloprotease-9 (MMP-9). severity. Aim: To identify novel urinary protein biomarker targets to facilitate development of a point of care testing device for monitoring LN disease activity.6 3.8-2.2. n¼2).6 3. Watson L.field extremity dedicated 0. 64(72356–65.6 1 Institute of Translational Medicine.1.8-2. receptor for advanced glycation end products (RAGE). Samples from JSLE patients (n¼4) participating in the UK JSLE Cohort study with active LN (renal domain British Isles Lupus Assessment Group (BILAG) score of ‘A or B’) were compared to age and sex matched healthy controls (HC.3. Caramella D.3-5.4 1. 15 RA patients (68%) and 9 H (18%). Lupus Downloaded from lup. Methods: SLE patients with arthritis (n¼50).8-3.3). Every patient underwent a non dominant hand (2nd–5th metacarpophalangeal joints) and wrist MRI without contrast injection with a low. 12(Suppl 10S63–8.4 2. 29(122635–42. annexin V.1 and Mosca M. Bombardieri S. Siena. Claude-Babron M.9th European Lupus Meeting 480 A080 and HSP. Distribution of joint erosions at the non –dominant hand and wrist in SLE. chemokine ligand 1 (CXCL1). No BME was found in H. Unfavorable outcomes (end-stage renal failure/death) in childhood onset systemic lupus erythematosus.1-5.2 2. resistin and tumor-necrosis-factor receptor-1 (TNFR1).1.5.1 1 Rheumatology Unit. 2 Hagelberg S. 3Department of Nephrolology. Great Ormond Street Hospital for Children Hospital.5 2. all RA and 47 (97%) H. Midgley A. Liverpool. prostate specific antigen (PSA). Clin Exp Rheum 1994. Erosions were observed at the hand in 24 SLE patients (48%).8-2.sagepub. Italy Introduction: Arthritis in systemic lupus erythematosus (SLE) is traditionally considered mild and non erosive. References 1 Watson L. 2014 .5 and Beresford M. Delle Sedie A. Pisa. Marks S.W.com at Institute of Marine Biology of Crete (IMBC) on October 26. UK. Further assessment in a larger. et al. Pilkington C. Vagnani S. Italy 3Radiology Unit. J Rheumatol 2002. similarly to RA.1. Alder Hey Children’s Hospital. Arth Rheum 2012. RA patients and in healthy subjects is reported in Table 1. Conclusions: This preliminary analysis has shown a number of novel urinary proteins to distinguish between patients with active LN. University of Pisa. Tullus K. D’aniello D.4 1.3.1-3. with between 4-44% progressing to end stage renal disease (2.3 1. Pisa. Cagnoni M. Long-term follow-up of childhood lupus nephritis. University of Liverpool. Leone V.1 2.1. et al. IL-1ra. London. A multicenter study in Paris and its environs. as compared with HC (Table 1). Department of Clinical and Experimental Medicine. hand disability and deformities are frequently reported by patients with longstanding disease. prospective paediatric cohort is warranted. and thrombospondin-1 as compared with HC. Conclusions: Joint involvement in SLE can be severe with a significant tendency to develop BME and erosions. University of Pisa. epidermal growth factor receptor (EGF R).5 1. and damage in the UK Juvenile-Onset Systemic Lupus Erythematosus Cohort. University of Siena.9-4.1 A081 MRI PATTERN OF ARTHRITIS IN SLE: A COMPARATIVE STUDY WITH RHEUMATOID ARTHRITIS AND HEALTHY SUBJECTS Tani C.6 1. Magnetic resonance imaging (MRI) has demonstrated to be more sensitive for identifying bone damage than conventional radiography and it is widely used as an outcome measure of joint damage in rheumatoid arthritis (RA) clinical trials. vascular endothelial growth factor (VEGF). Lee Y. at the wrist. Great Ormond Street Hospital. Possemato N.7 2.1.7 2.6 2.5 2. Objectives: In this study we aimed at describing the magnetic resonance imaging (MRI) pattern of distribution of bone marrow oedema (BME) and joint erosion in hands and wrists of patients with Systemic Lupus Erythematosus (SLE) with arthritis in comparison with Rheumatoid arthritis (RA) and healthy subjects (H). The cumulative erosive burden in SLE was significantly higher than in H (p¼0. Results: All JSLE patients (n¼4) with active LN displayed a 2-fold increase in the expression of chemokine ligand 16 (CXCL16). 4 Paediatric Rheumatology Department. 6 Department of Rheumatology. IDENTIFYING FUTURE URINARY BIOMARKER TARGETS FOR MONITORING OF LUPUS NEPHRITIS DISEASE ACTIVITY Smith E. Active LN patients displayed a higher expression of MMP9. annexin V and TWEAK than active HSP patients.1 2. and renal inflammatory control patients (Henoch-Schonlein-Purpura. Riente L. London.4-2. Carli L. 3 Levy M.0 3. Relative fold differences in urinary protein expression between JSLE patients and healthy controls Fold differences in urinary protein expression relative to health control Urinary Proteins Median fold difference Interquartile range CXCL16 MCP-1 Thrombospondin-1 TWEAK MMP-9 Annexin V EGF R CXCL1 PSA VEGF RAGE TNFR1 Resistin 2.1.002) but similar to RA patients.1-3. Bargman J. Liverpool.3-7. Results: BME was observed in 2 SLE patients at hand (4%) and in 15 at wrist (13%) versus 3 (30%) and 14(63%) RA patients. Montes de Oca M. 5 Nephrology Unit. Pilkington C.D. this condition is not rare as previously expected.5 2. monocyte chemoattractant protein (MCP-1). Jones C. Alder Hey Children’s Hospital. however chronic pain.1.2 Tesla instrument. HC Table 1. Italy 2GenOMEC PhD. patients with RA (n¼22) and H (n¼48) were enrolled.1.3.1.2 2.4 3.9-2.7 2. n¼2). UK. Disease activity.3 2. Introduction: Up to 80% of children with juvenile-onset systemic lupus erythematosus (JSLE) develop lupus nephritis (LN) (1). in 41 (82%) SLE. HSP.5 2.8-2.1. 75% of patients with active LN displayed a 2fold increase in the expression of: tumor necrosis like weak inducer of apoptosis (TWEAK).4. Liverpool. Patients and methods: The relative expression of 38 urinary proteins was analysed using a kidney biomarker proteome profiler array (R&D systems Ltd).1. 2014 .03 0. RT-PCR revealed significantly higher PF4var expression in platelets derived from APS patients comparing to healthy donors or lupus patients(2-CT: APS: 1.20[16..2 pg/ml versus HD: 60. Plasma levels of PF-4var were determined using a commercially available ELISA. Complete blood count. intarquartile range 65. PF-4var levels were significantly elevated in patients suffering from primary APS (PAPS) than those with APS secondary to SLE (SAPS) [p¼0.008 0. the major autoantigen in APS.04 ns ns ns ns ns ns ns ns ns ns ns ns 0.0017).006 0.37-172.03 ns ns 0. In addition.796] versus HD: 0. as indicated by p38MAPK phosphorylation and thromboxane production. School of Medicine. Platelet PF-4var and PF4 RNA levels were determined in RNA isolated from donor’s and patient’s platelet preparations using quantitative Real-Time PCR.0086]. Previous study from our group demonstrates a novel interaction between b2-glycoprotein I (b2GPI).132]. such as PF-4 and a recently isolated protein product of its nonallelic variant gene PF-4var. p<0.01 ns 0. anticardiolipin (anti-CL) and anti-b2GPI antibodies were measured. A NON ALLELIC VARIANT OF PLATELET FACTOR-4 (PF-4/CXCL4).6743-2.0497. Athens. respectively).4 pg/ ml. blood samples were taken and separate samples of serum. The precise role of PF-4var regarding the haemostatic balance is not yet studied.05 3 4 3 4 10 10 7 6 6 13 43 20 19 47 37 3 16 13 15 9 7 4 4 0. Objectives: To determine PF-4var plasma levels and platelet PF-4var expression (RNA level) in patients with APS and evaluate the correlation with clinical and laboratory parameters of the disease.0195. C-reactive protein (CRP).04 ns 8 8 5 1 9 12 4 9 10 7 19 12 13 21 20 2 12 6 9 11 11 6 1 A082 PLASMA LEVELS OF PF-4VAR/CXCL4L1. p¼0.461. Statistical analyses were evaluated by Mann-Whitney t-test and Kruskal-Wallis test. and Vlachoyiannopoulos P. This complex formation leads in the stabilization of b2GPI dimeric structure which facilitates the antibody recognition and platelet activation. Greece Background: Platelet derived chemokines. p¼0. Distribution of joint erosions at the non –dominant hand and wrist in SLE.136] p¼0. Methods: From 90 patients. The above chemokines present only 4.02 0. however they exhibit distinct platelet secretion mode and function. Patsouras M. ARE ELEVATED IN PATIENTS WITH ANTIPHOSPHOLIPID SYNDROME (APS) Sikara M. which absolutely discriminates PF-4var from PF-4 protein.7505 [0.3% aminoacid divergence in the mature proteins. (6%) (8%) (6%) (8%) (20%) (20%) (14%) (12%) (12%) (26%) (86%) (40%) (38%) (94%) (74%) (6%) (32%) (26%) (30%) (18%) (14%) (8%) (8%) (36%) (36%) (23%) (4%) (41%) (54%) (18%) (41%) (45%) (31%) (86%) (54%) (59%) (95%) (90%) (9%) (54%) (27%) (40%) (50%) (50%) (27%) (4%) A healthy control (n¼55) and a disease control group (SLE. who fulfill the revised diagnostic criteria for APS.69-219.9th European Lupus Meeting 481 Table 1.12] pg/ml. erythrocyte sedimentation rate (ESR). patients who experienced thrombotic events versus pregnancy morbidity or arterial versus venous thrombotic events do not show statistically significant difference in PF-4var levels.03 ns ns ns ns ns ns ns ns ns ns 0.sagepub.0027).47901. aPTT.9] pg/ml. [0.0020 or SLE: 0.. plasma and platelets were isolated.com at Institute of Marine Biology of Crete (IMBC) on October 26.01 0.0001 or SLE: 51. National University of Athens. Tzioufas A.003 0.4260-1.52 [36.04 ns 0. Dept of Pathophysiology. are implicated in several aspects of vascular thrombosis and inflammation. and PF-4 or PF-4var. A positive correlation was also revealed between the presence of thrombocytopenia and the elongation of aPTT with the higher PF-4var levels (p¼ 0. Conclusions: Preliminary results suggest that higher PF-4var levels are present in plasma of APS patients and especially in those with PAPS and these are associated with laboratory characteristics indicative for higher risk Lupus Downloaded from lup.815 [0. RA patients and in healthy subjects II metacarpal head III metacarpal head IV metacarpal head V metacarpal head II phalangeal base III phalangeal base IV phalangeal base V phalangeal base Trapezius Trapezoid Capitate Hamate Scaphoid Lunate Triquetum Pisiform Radius Ulna Basis MC I Basis MC II Basis MC III Basis MC IV Basis MC V H (%) P (SLE vs H) SLE (%) P (SLE vs RA) RA (%) 0 0 0 0 4 (9%) 3 (7%) 0 3 (7%) 4 (8%) 9 (19%) 32 (66%) 9 (19%) 10 (20%) 40 (83%) 28 (58%) 1 (2%) 9 (19%) 7 (14%) 4 (8%) 0 1 (2%) 2 (4%) 0 ns ns ns ns ns ns 0. n¼30) were included in the study. Regarding the clinical presentation of the disease.0048 and p¼0. Results: APS patients showed higher levels of plasma PF-4var compared to healthy individuals or SLE patients (APS: median 137. 0.017). anti-centromere in5. Gusetu G. Aim: To investigate in a cohort of SLE patients.1. Some of them are related to a distinct clinical subset of disease. Results: Disease activity was present in 10 (SLEDAI >3) and damage was present in 6 patients (SLICC index >0).1 1 Iuliu Hatieganu University. Cinar S.041). Turkey 2DETAE. standard cardiovascular risk factors and damage accrual in SLE patients. Brescia. CD40L and tPA levels were associated with serological and vasculitis items in SLEDAI.com at Institute of Marine Biology of Crete (IMBC) on October 26.2. Candrea E. Italy 2Spedali Civili of Brescia.Rheumatology and Clinical Immunology. CD40L levels were higher in SLE with damage(7474295365 vs 170918933 pg/ml. They were found in association with mucocutaneous and vascular features confirming previous reports(1) and mostly of these associations persisted after multivariate analysis. At multivariate analysis anti-Ki was significantly associated with male gender while anti-Ku with African ethnicity (P¼0.sagepub.050.016 NS.02 p¼0. anti-Ku in 8. 2014 . Cluj.2. References: Sherer Y. IL-8. Cartella S.032). Zdrenghea M.1. sE-selectin. Istanbul University. 3141823 vs 48781144 pg/ml.30. anti-PCNA and anti-Topo I in 3 each and anti-Jo-1 in 2 sera.2. No sign of muscular or pulmonary involvement was found in anti-Jo-1 positive patients while features of Systemic Sclerosis were detectable in 66% of anti-Topo I. Istanbul. tPA levels were higher in SLE with high vasculitis item in SLEDAI(n¼5. Taraborelli M. Patients and Metods: 20 SLE patients with RP fulfilling ACR and 72 SSc controls(49 limitted. but often silent in systemic lupus erythematosus (SLE). microhaemorrhages and increased CD40L were found to be related to damage. LV diastolic performance by mitral flow Lupus Downloaded from lup. Department of Internal Medicine.020) and lower in SLE with high serological item in SLEDAI(n¼7.1 and Rednic S. 80917050 vs 27821507 pg/ ml.Turkey Aim: We evaluate the relationship between semiquantitative scoring of nailfold videocapillaroscopy (NVC) and biomarkers in systemic lupus erythematosus (SLE) patients with Raynaud’s phenomenon(RP) and compared to systemic sclerosis(SSc) patients to investigate the pathogenesis of microvascular abnormalities. Clinical and serological features were collected from charts.1.1.5 vs 0. characterized by male gender and African origin. tPA. not significant Treatment and management of co-morbidities A085 A084 SEMIQUANTITATIVE CAPILLAROSCOPY ASSESSMENT AND BIOMARKERS IN SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS WITH RAYNAUD’S PHENOMENON: A COMPARATIVE STUDY WITH SYSTEMIC SCLEROSIS Yalcınkaya Y. Romania Introduction: Myocardial involvement is frequent. Serum samples of SLE patients and healthy controls were collected for flow-cytometric analysis of CD40L. Most of them are found as single anti-ENA specificity. Anti-Ki and anti-Ku are found in a subset of disease.1 1 Division of Rheumatology. Cavazzana I.7 and microangiopathy evolution score0. Ocal L.1. VEGF. p¼0. et al. echocardiography is a non-invasive imaging diagnostic method that effectively detects myocardial performance.017 andP<0. independently from their frequency. p¼0.2 and Inanc M. valve abnormalities.1.5. MCP-1. Irregularly enlarged capillaries and microhaemorrhages scores were higher in SLE with damage(1. Aim: To determine the frequency of diastolic dysfunction and assess its association with disease characteristics.2 1 Spedali Civili and University of Brescia. Comparison of Vascular Biomarkers between SLE patients with Raynaud’s and Healthy Controls CD40L(pg/ml) tPA(pg/ml) MCP-1(pg/ml) VCAM(pg/ml) Healthy Controls (n¼21) SLE (n¼20) 2462013051 24151279 907300 32311435 3438656415 44274089 1284523 41841330 NS p¼0.0001 respectively). sPselectin. p¼0.190. Italy Introduction: A high number of antinuclear antibody specificities can be detected in Systemic Lupus Erythematosus (SLE). p¼0. Anti-Sm. MCP-1. anti-U1RNP and anti-Ro/SSA were the more frequent specificities in our cohort.002). Clinical features of scleroderma were found only in patients with anti-Topo I. Patients and methods: Fifty-seven consecutive SLE patients underwent standard transthoracic echocardiography (chamber diameters. Kamali S.015. Romania 2Babes-Bolyai University.1. 3079947 vs 46571198. Results: 319(58.60. Other anti-ENA antibodies were found in 50 positive sera(15. Conclusions: antibodies to cellular antigens more rarely found in SLE are detectable in more than 15% of patients with anti-ENA antibodies. all NVC scores were lower. VCAM levels were lower in patients with capillary number score0.018 p¼0. VCAM levels were lower in late NVC findings like capillary loss and high microangiopathic score. SeminArthritisRheum2004. the prevalence and the clinical relevance of autoantibodies to cellular antigens less frequently found in SLE in our cohort of patients Patients and methods: Antinuclear antibodies were detected by indirect immunofluorescence on HEp-2 cells while counterimmunoelectrophoresis was used to detect anti-ENA antibodies in 540 patients. Cluj.1. tPA and VCAM levels were higher in SLE than healthy controls (Table 1). Brescia. Flestea A. 23 diffuse SSc) fulfilling Leroy&Medsger’s CLINICAL DETERMINANTS OF MYOCARDIAL DIASTOLIC DYSFUNCTION IN SYSTEMIC LUPUS ERYTHEMATOSUS Pamfil C. p¼0.1.3 criteria were investigated with NVC(Sulli A. IL-6. Rheumatology and Clinical Immunology. Table 1. Autoantibody explosion in systemic lupus erythematosus: More than 100 different antibodies found in SLE patients. Pearson chi-square/Fischer’s and Mann Withney U tests were used for statistical analysis. Artım-Esen B. Deniz G. Istanbul University. Tincani A.3 vs 0.06.1 and Franceschini F. p¼0. TGF-b and VCAM(Bender MedSystems. LVEF. 54% of these antibodies were detected as the single anti-ENA specificity. microhaemorrhage scores were similar to SSc.9%) out of 540 sera were positive for anti-ENA antibodies. et al). In SLE. Istanbul.Vienna) simultaneously with NVC. Anti-Ki antibodies were detected in 31. respectively.7 (n¼6. 124766756 vs 1143931299 pg/ml. Conclusion: The frequency of NVC abnormalities were lower in SLE except microhaemorrhages Irregular capillary enlargement. isolated anti-La/SSB. NVC is a promising tool for the investigation of vascular pathology in SLE and requires further investigation. p¼0.6%). Istanbul Medical Faculty.009 and n¼8.1.9th European Lupus Meeting 482 A083 RARE AUTOANTIBODIES TO CELLULAR ANTIGENS IN SYSTEMIC LUPUS ERYTHEMATOSUS Fredi M. Zdrenghea D.2. 7 while mean chronicity index was 1. mean  standard deviation age at SLE diagnosis and disease duration were 36  11.2.66(4):506–510. Mean activity index from the first to the fifth biopsy was 7. p<0. Among types of symptomatic cardiac involvement. et al. p¼0. osteonecrosis and/or cataracts) were compared between both groups.6% there was the exact opposite transition. p¼0. Cumulative organ damage and antiphospholipid antibodies.31 vs. in particular deep vein thrombosis (p¼0. Repeat renal biopsy in lupus nephritis: a change in histological pattern is common. Biopsies were blindly reassessed by two renal pathologists and were compared according to the newest ISN/RPS classification. Am J Nephrol 2011.4 vs. In 5.9th European Lupus Meeting 483 assessment and TDI of the mitral ring).9 years. 32%. and who were treated using different regimes combining medium-low dose prednisone (i. 4.16:2890-2894. References 1 Paran D. 1. and Ruiz – Irastorza G.037). Results: Within the cohort. Patients and Methods: We retrospectively analyzed laboratory parameters and therapeutic changes in 20 patients with lupus nephritis and repeat renal biopsies. baseline SLEDAI was similar in both groups (10 vs. 34: 220–225. whilst proliferative lupus nephritis was more frequent among patients in the high-dose group (12% vs. the evolution of the SLEDAI scores at year 2 was compared. The use of antimalarials and pulse methyl-prednisolone was more frequent among patients in the low-dose group (100% vs. Clinical and prognostic value of serial renal biopsies in lupus nephritis.019) and cumulative damage accrual (p¼0. osteoporotic fractures. cumulative clinical features. 4 Moroni G . 5. 5.. Likewise. The mean maximum prednisone dose received was significantly lower in the low-dose group (11 vs. 2014 .4 vs. respectively.05). patients in the low-dose group had a lower SDI score at year 5 (0. Athens. as measured by the SLICC damage index (SDI). Likewise. renal 20%.2 years and 9. et al. is still debatable. Nephrol Dial Transplant 2009. UPV/EHU Objective: To analyse the efficacy and toxicity of low-medium prednisone-based regimes to treat moderate-severe lupus activity. Greece 2Pathology Department. respectively. p¼0. 2. 63 mg/d. the frequency of prednisone-attributed damage at year 5 was lower in patients in the low-dose group (2% vs. p¼0. 0: 1–7. whilst there were no differences in the use of immunosuppressive drugs (34% vs.2.. damage at year 5. Methods: We selected from the Lupus-Cruces longitudinal cohort 41 patients presenting at the time of the diagnosis of SLE with moderatesevere lupus activity. These patients were matched according to their SLEDAI score with 41 historic controls who received high doses of prednisone at SLE diagnosis. 46%. p¼0.035). In order to compare the efficacy of both schemes. LESS TOXICITY Barbosa C. respectively.7. The mean SLEDAI at year 2 and the mean reduction in SLEDAI between baseline and year 2 were similar in both groups (4. Ann Rheum Dis. In order to analyse toxicity.5% there was conversion from proliferative to non proliferative class.. neuropsychiatric 21%.035). 7. 2 Lu J. 5 had four. 31 years. fourth and fifth biopsy was 28. Demographic data.5 vs. disease activity and damage scores were obtained. 24: 3712–3717. respectively. at least one cardiovascular risk factor was present in 40% of subjects but did not influence the development of diastolic dysfunction. A086 CLINICAL IMPACT OF REPEAT RENAL BIOPSIES IN PATIENTS WITH LUPUS NEPHRITIS Marinaki S. Results: There were no differences between both groups regarding gender distribution.04). 71% were female. Autoimmune diseases research unit. In 2. Liapis G. References 1 Daleboudt GMN. In 10.2% there were no findings of S. the most frequent being between classes III and IV (21%).004). Chinese Medical Journal 2012(125).9.E. 35. p¼0. p¼0.4.com at Institute of Marine Biology of Crete (IMBC) on October 26.36). 2007. Conclusions: Myocardial diastolic dysfunction is a frequent echocardiographic finding. Conclusion: Repeat biopsy is a reliable tool for monitoring the activity and chronicity status of the disease and for guiding therapeutic decisions especially late in the course of the disease. pulse-methyl prednisolone and/or immunosuppressive drugs.31). University of Athens Introduction: The clinical impact of repeat renal biopsies in patients with S. respectively. 12%. Ruiz – Arruza I.001).001. but not cardiovascular risk factors. Kapsia H. p¼0. Patients in the low-dose group were older at SLE diagnosis (38 vs.L. Diastolic dysfunction was associated with the presence of antiphospholipid antibodies (p¼0. respectively.1. autoantibody profile and baseline SDI.87. Importantly. Laiko Hospital.01). 5.1. The clinical relevance of a repeat biopsy in lupus nephritis flares.8). On the contrary.E.05) and a history of cardiovascular events. A087 LOW-MEDIUM DOSE PREDNISONE TO TREAT MODERATESEVERE LUPUS ACTIVITY: SAME EFFICACY. diastolic dysfunction was associated with antiphospholipid syndrome (p¼0.05). Medical School.N. 5 Abdulkareem A.4. 4 had five and 1 patient had six.6  8.9.L. 5.01) and a lower increase of the SDI score from baseline (0. nephritis. p<0. Median time between first and second. 24 mg/d.06). Results: Twenty SLE patients had two renal biopsies. and glucocorticoidattributed damage (defined as new diabetes mellitus. 27%. et al. 3 Wang G et al. 30 mg/d) with hydroxychloroquine. Lupus Downloaded from lup. and hematologic 10%.2. In 84% there was a change in therapeutical strategy after the repeat biopsy. 5. Strategy for second kidney biopsy in patients with lupus nephritis. A history of prolonged antiaggregant or anticoagulant therapy did not incur a protective effect against diastolic dysfunction..2. 0. Diastolic dysfunction was recorded in 37% of subjects and was associated with older age (p¼0.1 and Boletis J.sagepub. 4. cardiac 40%. Nakopoulou L. There was no correlation between laboratory parameters and the type of conversion. 8 had three.e. Skalioti C. 7. Histological conversion was observed in 58% of repeat biopsies. 9.9. Furthermore.7 respectively. either in means of increase (76%) or decrease (8%) of immunosuppression. et al. The presence of other severe disease manifestations or associated autoimmune diseases did not impact the diastolic dysfunction. diastolic dysfunction was associated with endocarditis (p¼0. Ugarte A. but not with SLE-related autoantibodies. Nephrol Dial Transplant 2011. defined as a SLEDAI score  6. Hospital Universitario Cruces. Sinodinou I. Aim: This retrospective analysis aimed to assess the hypothesis that repeat biopsy is essential for determining the activity and chronicity status of the disease and is a reliable tool in guiding therapeutic decisions. 10. 34% vs. third and fourth. second and third. 22%.03). with anticardiolipin antibodies (p¼0.1. Major/severe organ involvement within the cohort was as follows: cardiac 40%. 33 and 28 months respectively.1 1 Nephrology Department and Renal Transplantation Unit. Am J K idney Dis 1999. p¼0. 34: 530–539. In 21% there was a transition to a more severe form of nephritis. et al.5 vs. are major determinants for diastolic dysfunction in SLE patients. p¼0. Changes in pathological pattern and treatment regimens based on repeat renal biopsy in lupus nephritis.3.2. p<0.001) as well as the average daily prednisone dose received during the first year (4. 5%). 11(8533–5. Aim: Evaluation of treatment and renal outcome of RPGN in lupus patients with the use of repeat renal histology. 4 Chen S. who had the most severe chronic lesions already at the time of initial biopsy. especially glomerulosclerosis while 2 patients developed secondary FSGS.1 1 Nephrology Department and Renal Transplantation Unit.2.5 3 2.sagepub. Conclusions: The frequency of RPGN in lupus patients is low.com at Institute of Marine Biology of Crete (IMBC) on October 26. pulses of cyclophosphamide (0. Rapidly progressive lupus glomerulonephritis and concomitant microangiopathy in an adolescent. Greece Introduction: Proliferative lupus nephritis (LN) has a worse outcome and while crescents are common.8 5. et al.6 months from the initial biopsy. Patients and Methods: In a series of 195 renal biopsies performed in lupus patients during 2000-2013.v. However. In two large series of lupus patients from Far East the incidence of RPGN in LN is 3. Gakiopoulou X. A protocol renal biopsy was performed in a median time of 19.3 3 6 96 83 87 33 97 8. Am J Nephrol 2013. References 1 Sumethku V.5%. Liapis G. Kapsia H. Greece. All patients were treated with 3 daily i. Immunoadsorption sessions (6-8) were also applied. Clinical spectrum of diffuse crescentic glomerulonephritis in Chinese patients. Significance of histological crescent formation in patients with diffuse proliferative lupus nephritis.1. 116(111731–1740.8 0. 3 Tang Z. et al. Athens. 9: 424–428. aged 21 to 54 years. Medical School. Lupus 2009. RPGN was observed in 5 (2.N.5-1g/24h) and 6 monthly i. 2 Blake JS.5-1g/m2) in combination with the anti-CD20 monoclonal antibody Rituximab (1g/2weeks).1. Lupus 2002.6 2. is as effective and has less associated toxicity than high dose prednisone-b A088 TREATMENT AND RENAL OUTCOME OF RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS IN LUPUS PATIENTS Marinaki S. Histological findings at initial biopsy Tubular SLE Activity Chronicity Atrophy/Interstitial Glomerulosclerosis Class Index Index Crescents (%) Fibrosis (%) (%) 1 IV 20 2 IV 17 3 IVþV 14 4 IV 14 5 IV 21 4 6 5 6 0 77 70 50 53 53 10 35 10 40 10 Three of them were men and 2 women. Renal function and proteinuria during follow-up time At 6th month At initial Biopsy At time of Protocol Biopsy At end of follow-up eGFR (ml/min) Proteinuria (g/24h) eGFR (ml/min) Proteinuria (g/24h) eGFR (ml/min) Proteinuria (g/24h) eGFR (ml/min) Proteinuria (g/24h) 1 2 3 4 5 33 38 39 22 26 3 7. All protocol biopsies revealed a substantial increase in chronic lesions.1 and Boletis J.92 1 4 0. rapidly progressive glomerulonephritis (RPGN) is rather rare and not clearly distinguished from the proliferative classes. Skalioti C. Laiko Hospital. 38(6445–52. et al. Therefore.8 Lupus Downloaded from lup. 2Pathology Department. University of Athens. Lupus nephritis: a challenging cause of rapidly progressive crescentic glomerulonephritis.15 1.2.v. Results: Histology and laboratory parameters are presented in Tables 1-3 and were examined from the time of initial biopsy until the end of follow-up (12/2013). Table 1. combined with hydroxychloroquine.15 0. pulses of methylprednisolone (0.9 111 104 95 27 111 10 1 0. e GFR had a tremendous improvement in all but one patient (No 4). Histological findings at protocol biopsy 1 2 3 4 5 Time from initial biopsy (months) SLE class Activity Index Chronicity Index Other Histological Findings Tubular Atrophy/Interstitial Fibrosis (%) Glomerulo-sclerosis (%) 12 19 22 24 21 III III - 3 0 - 8 5 - FSGS FSGS 25 35 10 50 10 48 45 21 58 21 Table 3. data regarding the therapy and clinical outcome of RPGN in lupus patients is scarce. Intensive therapy leads to rapid and long-standing improvement of renal function although it does not prevent the progression of histological chronic lesions. Chin Med J(Engl) 2003. with proliferative LN (III or IV) and >50% crescent formation in the initial biopsy. pulsemethyl prednisolone and/or immunosuppressive drugs.8 0.9th European Lupus Meeting 484 Conclusion: The use of therapeutic regimes containing low-medium doses of prednisone. 2014 92 81 94 28 131 0. 5 11 15 25 12 Table 2. et al.3 . Moser K. Petri M. Moser K.1. Stockholm University.2. Strand V.2 1 Department of Rheumatology and Clinical Immunology. Svenungsson E. Berlin. In the patients with former LN. USA 4UCB Pharma. Charite´ University Hospital Berlin. We observed no significant increase in C3/C4 levels. Birmingham. Brussels. Hoyer BF. No severe adverse events were noted. Johns Hopkins University. Patients and Methods: Fourteen patients were included.2 and Hoyer B.2. Karolinska Institutet.E. and 1 cyclosporine. 3 Mumtaz IM. USA 6Biopharmaceutical Consultant. It is the only biologic agent approved for treatment of Systemic Lupus Erythematosus (SLE).9th European Lupus Meeting 485 New therapies. before week 8 and before the onset of symptoms.1. Persistent depletion of LLPCs was achieved only by combining a shot of bortezomib with maintenance therapy. Durham. Despite the limited number of patients.01-0.3.J.. Los Angeles.A.2 g/day. no renal flare was observed during the observation period. range 0. Mei H. autoreactive LLPCs are generated throughout life. Berlin. thereby reducing autoantibody levels. it is unknown whether their generation continues in the established disease. 2 Hoyer BF. 7(3170–178.003) and 52 (p¼0. A090 DECREASED SLE DISEASE ACTIVITY AND CORTICOSTEROID USAGE AND NO RENAL FLARES DURING BELIMUMAB TREATMENT Parodis I. Kilgallen B.1. UK 9Cedars-Sinai Medical Center. Belgium 3School of Medicine. respectively). Voigt C. respectively) and 52 (p¼0. Conclusions: Belimumab treatment decreased SLE disease activity and reduced corticosteroid usage. The long-lived plasma cells (LLPC) secreting such autoantibodies are refractory to conventional immunosuppressive treatments [2.3. Radbruch A. Cheng Q. Lupus Downloaded from lup. LLPC counts in the spleen plateaued by week 10.1 1 Department of Medicine. skin involvement (n¼11). range 0.001 and p¼0. Short-lived plasmablasts and long-lived plasma cells contribute to chronic humoral autoimmunity in NZB/W mice. Their sustained therapeutic elimination requires both the depletion of LLPCs and the inhibition of their regeneration. University of Luebeck. Khodadadi R.01-0. all patients received oral prednisolone (mean dose 11. The median SLEDAI and SLAM scores were 10 (range 2-24) and 12 (range 5-23). respectively. Gordon C. USA 5UCSD School of Medicine. 39(3180–188. Results: At baseline. Long-lived autoreactive plasma cells drive persistent autoimmune inflammation.1.sagepub. we analyze the generation of autoreactive LLPCs in lupusprone NZB/W F1 mice over their lifetime. Clinical data were acquired at baseline and week 12. Cheng Q. Dorner T. Sweden Introduction: Belimumab is a recombinant monoclonal antibody that specifically binds to soluble B-lymphocyte stimulator.14 g/day.028 and p¼0. but continued to increase in the bone marrow.B. epratuzumab (a monoclonal antibody targeting CD22) produced clinically relevant improvements in disease activity in patients with moderate-to-severe systemic lupus erythematosus (SLE).1.5. Journal of Autoimmunity 2012 Sep. Material and methods: Bromodesoxyuridine pulse chase experiments were used to analyse the kinetic of the development of the LLPC compartment in lupus-prone NZB/W F1 mice. Luebeck. Eilat D. novel targets of therapy and regulatory issues A089 AUTOREACTIVE LONG-LIVED PLASMA CELLS IN NZB/W MICE AND THEIR REGENERATION Taddeo A. Hiepe F. Hoyer BF. The grade of proteinuria remained unchanged at week 26 (median 0.5 g/day) and 52 (median 0. 9 received antimalarials. et al. The Journal of experimental medicine 2004 Jun 7.008). et al. Stockholm.1. Brussels. The generation of LLPCs then continues throughout life. Although they are generated long before the disease becomes clinically apparent. USA Introduction: In the EMBLEMTM 12-week dose-ranging phase IIb study. Nature reviews Rheumatology 2011 Mar. depleting the precursors of LLPCs or preventing their differentiation into LLPCs. Kalunian K.com at Institute of Marine Biology of Crete (IMBC) on October 26.1.13 g/day. Ten patients had low complement at baseline. Panne D. Hauser AE. Nine patients with former LN were included (median proteinuria at baseline 0. 2 azathioprine. a Leibniz Institute. Hauser AE.007. Stockholm. involvement of the central nervous system (n¼4) and cytopenias (n¼3). Radbruch A .g. Aim: To investigate the effects of belimumab given as an add-on to patients with active SLE despite standard-of-care therapy. Portola Valley. 199(111577–1584. Disease activity was assessed using SLE Disease Activity Index 2000 (SLEDAI-2K) and Systemic Lupus Activity Measure (SLAM). Germany Introduction: Autoantibodies contribute significantly to the pathogenesis of systemic lupus erythematosus (SLE) [1]. References 1 Hiepe F. One patient withdrew due to allergic reaction. 3]. 26 and 52.F.24 g/day). When LLPCs are depleted by the proteasome inhibitor bortezomib. Winter O.9 1 Duke University Medical Center. Germany 2German Rheumatism Research Centre. Baltimore.02-0. Rheumatology Unit.4. Peddinghaus A. range 5-20 mg/day). Significant decreases of SLAM and SLEDAI were seen at week 26 (p¼0. USA 2Universite´ Catholique de Louvain. USA 7UCB Pharma. Bongardt S. and LLPC regeneration after depletion. range 0. LLPC were anylsed using flow cytometry and ELIspot technique. Raleigh. Depletion was performed using bortezomib and cyclophsphamide. The primary organ manifestations comprised arthritis (n¼9). 2014 . Axelsson M. Conclusion: In lupus-prone NZB/W F1 mice.8 and Wallace D.012. their numbers regenerate within two weeks. serositis (n¼3). Results: Autoreactive LLPCs are established in the spleen and bone marrow of lupus-prone mice very early in ontogeny. La Jolla. with focus on low-active lupus nephritis (LN) patients. cyclophosphamide. Houssiau F. Sweden 2AlbaNova. Bone marrow of NZB/W mice is the major site for plasma cells resistant to dexamethasone and cyclophosphamide. Prednisolone dosages were significantly decreased compared to baseline at week 26 (p¼0.2.1.7. our observations indicate that belimumab may prevent renal flares and may be used in patients with former LN and persistent A091 EPRATUZUMAB MAINTAINS IMPROVEMENTS IN DISEASE ACTIVITY FOR OVER 2 YEARS IN PATIENTS WITH MODERATE-TO-SEVERE SYSTEMIC LUPUS ERYTHEMATOSUS Clowse M.6.4 g/day). 3 mycophenolate mofetil. Aim: Here. Manz R. Germany 3Institute for Systemic Inflammation Research.2 and Gunnarsson I. e. Belgium 8University of Birmingham.2. BILAG. Patients and Methods: EMBLEMTM patients who completed 12 weeks’ blinded treatment or discontinued after 8 weeks due to lack of efficacy were eligible for the OLE.0% in the musculoskeletal.5 25. n Any AE Related to study drug Causing discontinuation Serious AEs SLE flare Lupus nephritis Symptomatic cholelithiasis Infections/infestations Urinary tract infection Upper respiratory tract infection Sinusitis Infusion reactions Deaths 192 87 29 57 7 4 3 138 50 47 22 29 1 94.3. Spain 3East Bay Rheumatology Medical Group.8%. mucocutaneous. cardiorespiratory. USA Introduction: In the EMBLEMTM 12-week dose-ranging phase IIb study. Birmingham. with no new safety signals identified.3% met combined treatment response criteria (Table 1).8% and 10. Pike M.9 14. University Hospital No. Consistent BILAG. Johns Hopkins University.Proportion of patients requiring CS 7.7 25. Endpoints included BILAG improvement. % Events. Bydgoszcz. Raleigh. 35 (17%) and 168 (83%) received placebo and epratuzumab (various doses) respectively in EMBLEMTM. neuropsychiatric.11 1 Cedars-Sinai Medical Center.1 41.com at Institute of Marine Biology of Crete (IMBC) on October 26.3 57.9th European Lupus Meeting 486 Table 1. Results: In the OLE. Poland 10 University of Birmingham.0 (6–39) 50. USA 8Harvard Medical School. Median CS dose was 10. 36. USA 6UCB Pharma. USA 2Hospital Vall d’Hebron. There were no opportunistic infections. Observed data are reported to OLE Week 108.0 (0–30) 18.6 23.3 56.1 3. no SLEDAI worsening.sagepub. USA. Lupus Downloaded from lup.5 62. adverse events (AEs).4% had no worsening in SLEDAI and PGA respectively.0 (12–61) 12. Neuwelt M.0 1. Baltimore. Barcelona.J. n Patients.0 (0–72) 8.7 33. La Jolla. Median BILAG total score decreased by 64%. Kalunian K. Aim: To evaluate the long-term safety profile and effect on corticosteroid (CS) use of epratuzumab treatment in patients with moderate-tosevere SLE.and PGA scores from EMBLEMTM baseline through to Wk 108 and last visit All patients Combined Treatment Response (%) EMBLEMTM placebo patients (%) EMBLEMTM epratuzumab patients (%) BILAG median (range) SLEDAI median (range) PGA median (range) EM BLEMTM baseline OLE screening Week 48 Week 96 Week 108 Last Visita 32.8 14.9 55.0 (0–34) 31. 34.0 (0–32) 25. 2.1% and 52.6 45.0% at Week 116. receiving 1200mg epratuzumab at Weeks 0 and 2 of 12-week cycles with efficacy evaluated at Weeks 4 and 8.0% and 97.6. no PGA worsening relative to baseline). Combined treatment response.2 58. in disease activity in patients with moderateto-severe SLE. USA 4UCSD School of Medicine. Belgium 7School of Medicine. Physician Global Assessment (PGA) score and combined treatment response (BILAG improvement without worsening. Table 1.0 (0–94) a Last visit ¼ last available value for each subject regardless of timing Aim: To report long-term efficacy of epratuzumab from the open-label extension (OLE) (SL0008) of EMBLEMTM (NCT00660881). Jeka S.5mg/day.4. and 5.9%]) infections.0%.0 (0–96) 9.0 (0–52) 6. Patients and Methods: EMBLEMTM patients who completed 12 weeks’ blinded treatment or discontinued after 8 weeks due to lack of efficacy were eligible for entry into an open-label extension (OLE) study.2 10.7 38.0 (0–81) 9. Ordi-Ros J.0 (0–24) 17. CS doses were converted to prednisone equivalents.4 2.0 (0–57) 10.7 40.10 and Strand V.1 40. receiving 1200mg epratuzumab at Weeks 0 and 2 of 12-week cycles with efficacy evaluated at Weeks 4 and 8. 41. Bongardt S. Safety variables included exposure duration.2. median (range) epratuzumab exposure was 845 (75–1185) days across 203 patients.3 0. no patterns of specific serious (n¼14 [6.8. One patient died of cardiac failure.0mg/day at Week 116 (n¼112). 2014 .5 1946 300 35 98 7 4 3 487 87 93 30 74 1 A092 EPRATUZUMAB: SUSTAINED LONG-TERM SAFETY PROFILE AND EFFECT ON CORTICOSTEROID USE IN PATIENTS WITH MODERATE-TO-SEVERE SYSTEMIC LUPUS ERYTHEMATOSUS Wallace D.0 (0–22) 19. SLEDAI. Category Patients. Harvard.3 28. and 60.6 56.0 24. At Week 108. and no indication of an increased risk of malignancies.2 57.5 60. epratuzumab (a monoclonal antibody targeting CD22) was safe and effective in the treatment of patients with moderate-tosevere systemic lupus erythematosus (SLE).9 41. Conclusions: Long-term treatment with epratuzumab was associated with decreases in CS use in those receiving >7. Petri M.8% at OLE entry. Conclusion: Epratuzumab was associated with sustained improvements.0 (9–90) 14. USA 5UCB Pharma. respectively).5. SLEDAI assessment score.6 42.5 (0–69) 10.6%. 84.0mg/day at EMBLEMTM baseline and OLE entry. Data are summarized using descriptive statistics. Kilgallen B. constitutional and renal body systems respectively). SLEDAI and PGA improvements were observed between EMBLEMTM baseline and OLE Week 108 (Table 1). Gordon C. 94. Epratuzumab had an acceptable safety profile. Improvements from BILAG A/B to BILAG C or D were observed across body systems (44.9% and 8. infusion reactions/infections.0 (0–52) 4. Brussels. beyond 2 years. Portola Valley. Los Angeles.7. UK 11Biopharmaceutical Consultant.9.0 61. AEs are summarized in the Table 1. Results: 203 patients received a median 845 (range:75–1185) days’ epratuzumab exposure.1.0 (0–52) 5.5 68.7%.5–20mg/day and >20mg/day decreased (49.0 (0–73) 9. San Leandro. 33. USA.0 (9–727) 116. 6Biopharmaceutical Consultant.0 (11–1122) 142.5 (13–415) 80. Tcell counts remained stable (2 years in SL0008. CD3þ T-cell counts and IgG/IgA/IgM levels were measured.4.1 (-75–220) -36. Peripheral blood CD19þ B-cell counts.6.0 (10–818) 149. the SL0006 open-label extension (OLE) utilized 360mg/m2 epratuzumab.5 (3–508) n 40 11 51 37 37 Week 12 59.9th European Lupus Meeting 487 A093 IMMUNOLOGIC RESPONSE TO EPRATUZUMAB IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS Petri M.M.8. Belgium.0 (6–1002) 140.7 (–88–313)* % (range) –46.1 (–81–219) % (range)* –49.0 (0–526) % (range)* –45. patients received placebo.5 (20–586) Week 48 70. Aim: To report immunologic response in short-term and long-term epratuzumab trials.9 (-75–40) -14. Kilgallen B.5 (10–1814) 134.0 (10–1291) 109. B-cell counts were reduced from baseline in ALLEVIATE and EMBLEMTM (Table 1). 9Hospital San Lucas Da Pucrs.2 (–99–1767) n 25 n 195 Week 48 56. patients entering the OLE (SL0008) received 1200mg epratuzumab at Weeks 0 and 2 of repeating 12-week cycles.5.7.5 (20–394) Week 96 60.3.0 (20–460) Week 112 59. Baltimore. Morris Plains.2 (-75–126) -28. Los Angeles.12.6 (-58–1767) n 29 34 10 44 30 28 SL0006 360 mg/m2 32 26 Screening V1 41. Portola Valley.3 (-99–85) -15.0 (–94–286) n 21 n 106 Week 192 48. Boston. Poland.J. San Diego. Table 1. Brussels. Morris Plains. 14Center for Molecular Medicine and Immunology. 12UCB Pharma.sagepub.–30% to –40% at most timepoints thereafter).0 (–100–215) n 28 n 142 Week 96 63.3 (-75–122) -26. USA. this decrease was maintained throughout SL0008.0 (16–474) % change (range)* -34.2. Patients and Methods: In the ALLEVIATE-1/2 trials. 8Jozef Strus Municipal Hospital.6 (–84–462) n 18 32 33 SL0008 1200 mg * Median (range) percent change in B-cell counts from baseline Lupus Downloaded from lup. In EMBLEMTM. CD22 expression rapidly decreased (around 80% of baseline by Week 4).com at Institute of Marine Biology of Crete (IMBC) on October 26. Leszczynski P. Bongardt S.0 (1–615) 100.13 and Goldenberg D. produced clinically-relevant improvements in disease activity in moderate-to-severe systemic lupus erythematosus (SLE) patients in the EMBLEMTM study. Belgium.0 (10–1529) 104.11.1 (-92–145) -55. 4Harvard Medical School. Houssiau F. In SL0008. Median (range) absolute B-cell counts (cells/mL) and median percent (range) change from baseline in B-cell counts during short and long-term SLE treatment with epratuzumab Median (range) absolute B-cell counts (cells/mL) EMBLEMTM ALLEVIATE 360 mg/m2 720 mg/m2 All active Placebo 600 mg weekly 100 mg EOW 400 mg EOW 1200 mg EOW 1800 mg EOW Placebo Baseline 99.5 (27–362) 60. 11Cedars-Sinai Medical Center. 4 years in SL0006). Strand V. Birmingham.9 (-86–770) -19.0 (–93–325) % (range)* –50.A. B-cell surface CD22. Gordon C.9 (-92–220) 1.0 (3–274) % (range)* –48. UK. patients received 12 weeks placebo or epratuzumab (dose-ranging up to 3600mg cumulative dose). 3University of California. USA.4 (–97–244) n 22 n 111 Week 144 51.0 (10–1814) 53.0 (6–379) % (range)* –51. Wallace D. 2014 .10.5 (14–558) 91. 10 Immunomedics Inc. USA. 13UCB Pharma.0 (10–946) 125. Conclusions: B-cell counts were moderately reduced after 12 weeks epratuzumab treatment. USA.5 (19–931) 94. CD27-/IgD-/CD95þ B cells (a memory B-cell subset elevated in SLE flares) were also reduced. stabilising between -50% and -65% at most timepoints from Week 72. 2University of Birmingham.5 (20–449) % (range)* –66. Poznan. Brazil.0 (10–659) 60. USA. 360mg/m2 or 720mg/m2 epratuzumab in 12-week cycles up to 48 weeks. USA. Wegener W. Decreases continued into OLEs.9.0 (1–615) % (range)* –37.0 (20–1303) Screening V1 95. median IgA/IgG levels decreased minimally from baseline.7 (–89–357) % (range)* –17. Kalunian K.0 (17–533) 94. USA Introduction: Epratuzumab (a monoclonal antibody targeting CD22). Long-term treatment was associated with profound decreases in B-cell CD22. Results: After 12 weeks epratuzumab treatment. Raleigh. Porto Alegre.10.6 (-79–73) -14. Median IgM levels decreased (SL0006 year 1 –29% of baseline. 7Poznan Medical University.14 1 The Johns Hopkins University. 5Universite´ Catholique de Louvain.5 (10–659) 86. Pike M. Keiserman M.1.0 (18–465) 34 39 34 37 36 111. 4 10.4 and Gordon C.1. 2014 .9 63.1. Results: Remission occurred in 9 of 12 patients (75%) in the hUCMSC group and 5 of 6 patients (83%) in the placebo group.6 156 46. Aim: To report HRQoL data from EMBLEMTM and its open-label extension (OLE) (NCT00660881).1 12.3 12.5 56.USA. 5 University of Birmingham. or 2400mg cd as 600mg every week. Regnault A.2 and Lim T. Data are reported to the end of EMBLEMTM and OLE Wks48 and 108.3 8. 4Mapi HEOR & Strategic Market Access. Patients completing 12 weeks or discontinuing due to lack of efficacy after 8 weeks were eligible for OLE entry. All patients received standard immunosuppressive treatment which consisted of intravenous methylprednisolone and cyclophosphamide as induction.4 17. HRQoL was assessed using Short Form-36 (SF-36) v2.6 10.1 6. One patient on hUC MSC had leucopenia. Kilgallen B.5 156 40.9 203 58.5 Bodily pain 201 36. potentially due to short-term treatment. proteinuria. SF36 scores and MCID with epratuzumab in EMBLEMTM and EMBLEMTM OLE EMBLEMTM baseline EMBLEMTM OLE screening Week 48 of the EMBLEMTM OLE Mean change Mean absolute from EMBLEMTM Score baseline N Week 108 of the EMBLEMTM OLE Mean change from EMBLEMTM Mean absolute Score baseline N Mean change Mean absolute from EMBLEMTM % pts with Score baseline improvement  MCID Mean absolute Score N Physical functioning 201 47.1 203 58.7 Role emotional 201 50.5 1 Biopharmaceutical Consultant.1.0 113 40.0-points in domain and 2. This Phase 2 trial was based on Simon’s optimal two-stage design which requires 12 patients be enrolled in the first stage and an additional 24 patients in the second stage if >¼7 patients were to respond to hUC-MSC in the first stage (and the response rate were to exceed that of the parallel placebo control group).4 70. SLE Research Centre Yunnan China 2Sabah Medical Centre Sabah Malaysia 3ClinResearch SB Malaysia SUSTAINED IMPROVEMENTS IN HEALTH-RELATED QUALITY OF LIFE IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS FOLLOWING EPRATUZUMAB TREATMENT: RESULTS FROM A PHASE IIB TRIAL AND ITS OPEN-LABEL EXTENSION Strand V.4 113 40.1. 2Oklahoma Medical Research Foundation. pneumonia and subcutaneous abscess from which she recovered.5 11. renal function. Lyon. Introduction: Improvements in health-related quality of life (HRQoL) with epratuzumab (a monoclonal antibody targeting CD22) in systemic lupus erythematosus (SLE) patients have been reported in the ALLEVIATE trials from Week (Wk)12 and sustained to 4 years.9 114 61.5 11. Remission was defined as stabilization in renal function.5 13.8 16.1 156 61. urinary activity.5 73.2 14.L. Clinically meaningful changes vs EMBLEMTM baseline were observed in the OLE (Table 1).1 114 52. Raleigh.0 3.T. <10 urinary RBC/hpf and >50% reduction in proteinuria or <1g/day for subnephrotic proteinuria or to <3g/day for nephrotic range proteinuria at baseline. In the EMBLEMTM study (dose-ranging phase IIb randomized controlled trial [RCT] in SLE). Zhang P.8 7. with greater increases in the PBO group.0 17.1 156 63. OLE observed case analyses are presented.4 66. 17% of patients in both the hUC-MSC and placebo group achieved complete remission.5 Vitality 201 34.3 156 53. France. UK.4 General health 201 32.1 113 54.9 5. Conclusion: hUC-MSC has no apparent additional effect over and above standard immunosuppression.8 203 57. Tseu F. Portola Valley. Table 1. USA.GOV IDENTIFIER: NCT 01539902) Deng D.1 156 53. Nikaı¨ E.1 203 38.8 8. ANA.6 113 60. receiving 1200mg epratuzumab at Wks0 and 2 of 12-week cycles.3.com at Institute of Marine Biology of Crete (IMBC) on October 26.5 114 57.3 Role physical 201 37. Background and Aims: The aim of the study was to evaluate the efficacy of allogeneic Human Umbilical Cord derived Mesenchymal Stem Cell (hUC-MSC) for the treatment of proliferative lupus nephritis.6 Mental health 201 50.4 12.4 66.7 59.1 13.9 7.9 68. Mean changes and %pts reporting improvements minimum clinically important differences (MCID) (increases from baseline5.3.6 69.3 1 Department of Dermatology 2nd Affiliated Hospital of Kunming Medical University.2.9th European Lupus Meeting 488 A094 A095 A PHASE 2 TRIAL OF ALLOGENEIC HUMAN UMBILICAL CORD DERIVED MESENCHYMAL STEM CELL FOR THE TREATMENT OF LUPUS NEPHRITIS (CTS.0.3 203 42. Conclusions: No differences in improvements in HRQoL were observed with epratuzumab and PBO in the EMBLEMTM study.2 82.6 6.USA. Merrill J. MCS and domain scores across all groups generally increased from EMBLEMTM baseline through Wk12.7 16. or cumulative dose (cd) epratuzumab (200/ 800/2400/3600mg) as 2 equal every other week infusions. Results: Mean SF-36 PCS.1 10.9 156 61. Methods: In EMBLEMTM.4 PCS 201 36. Methods: Eighteen patients with WHO Class IV lupus nephritis were randomly assigned to either hUC-MSC (dose 2X108 cells) or placebo.3 13. epratuzumab treatment resulted in clinically relevant improvements in disease activity.0 203 56.O.5-points in physical and mental component scores [PCS and MCS]) are presented.6 114 47.9 203 51. Another had severe pneumonia which she died of.sagepub. anti-dsDNA. Oklahoma. small sample sizes and background therapy.7 16. SLEDAI and BILAG score were similar in both groups.8 MCS 201 36.7 4. 3UCB Pharma. Improvements in serum albumin.5 15.5 156 61.6 203 41.5 67.6 8. serum complement.3 SF36 domain N Lupus Downloaded from lup. The study was motivated by previous reports that showed dramatic response to hUC-MSC in patients with severe refractory SLE. followed by maintenance oral prednisolone and mycophenolate mofetil. Birmingham.5 113 60.1 203 48.3 17.7 Social functioning 201 46.1 203 40.1 113 43. One patient on placebo had a stroke and another had ascites of unknown cause. patients with moderate-to-severely active SLE (1 BILAG 2004 A or 2 Bs) were randomized to 1 of 6 12-week intravenous regimens plus standard of care (SOC): placebo. Long-term improvements in the OLE were consistent with those in the ALLEVIATE RCTs. Guo Y.7 156 46.9 156 41. DMARD’S: Disease Modifying Antirheumaticdrugs.Brescia. SjS: Sjo¨gren Syndrome.0%) and renal failure (23. Patients and Methods: We followed 36patients. (RANGE) ADDITIONAL RISK FACTORS TYPE OF DIAGNOSED INFERTILITY CHRONIC THERAPIES THYROID ALTERATIONS AUTOANTIBODIES INHERITED THROMBOPHILIA ART ATTEMPTS FAILED IN THE PAST 27 INFERTILITY COUNSELING 9 FOLLOWED PREGNANCIES 2SLE.1(11%) by Artificial Insemination.5.1SS.5%): Single:15(68%) Multiple:7(32%) primary:25(93%).3%) of patients (catheter related problems 6 patients..9%) and renal (63.Department of Obstetrics and Gynecology.1.1(11%) OocyteDonation.6%) of patients..0%) and dermatologic (3. Fifteen of the patients (25%) had TPE required disease involvement as presenting feature.5% of pregnancies: 2IUGR(Intrauterine Growth Table 1.com at Institute of Marine Biology of Crete (IMBC) on October 26. Levothyroxin(20%) n¼9(33%) n¼25(93%) single:44%. Results: The 9 prospectively followed pregnancies showed:-6(67%) were induced by FIVET (In Vitro Fertilization Embryo Transfer).4%) patients had vasculitis. 2(22%) LDA.38FIVET.3OocyteDonation 3SLA. Indications for TPE were hematologic (28.75%hidiopatic secondary:1(11%) n¼9(100%): LDA(44%).Brescia. AP: Anchylosing Spondylitis. respectively). myopathy (5.1PAPS.5. 1(11%) by IUI (Intrauterine Sperm Injection).-No thrombosis nor disease flares during gestations.leucopenia. Lojacono A.0%) patients had SLE.4%). PAPS: Primary Antiphospholipid Syndrom. Hacettepe University Faculty of Medicine. Indications for TPE. 14 (23. 1AS.sagepub. SS: Systemic Sclerosis.3%).9th European Lupus Meeting 489 A096 THERAPEUTIC PLASMA EXCHANGE (TPE) FOR REFRACTORY AUTOIMMUNE DISEASES REPORT OF 60 CASES Kiraz S. Family planning.2UCTD. Ankara Turkey Introduction: Therapeutic Plasma Exchange (TPE) is an effective therapeutic option for treating serious manifestations of systemic autoimmune diseases. Andreoli L. and Ertenli I..8%. 2 thrombocytopenia). Italy 2Spedali Civili and University of Brescia Maternal-Fetal Medicine Unit.2 and Tincani A.PDN(44%) n¼2(22%) n¼8(89%) single:22%.multiple:66% aPL:48% 53%(single:75% multiple:25%) n¼56 15AI.27for preconception counseling and 9pregnancy induced by assisted reproductive techniques (ART) (Table 1).[39].an increasing number of women ask for the evaluation of asymptomatic positivity for autoantibodies found in the workup of the centers for the diagnosis and the treatment of infertility. Karaagac T. Kalyoncu U. Karadag O.. Outcome analysis had been done in 55 (91.multiple:44% 25%(single:100%) n¼2 2AI aPL:44% Lupus Downloaded from lup. 1Takayasu.4%) causes. All patients were receiving corticosteroids at varying doses and all received a concomitant immunosuppressive drug.4%).6%) causes as TPE indication died whereas in neurologic and hematologic causes mortality were lower (26.1 1 Spedali Civili and University of Brescia. hypotension 3 patients.1 Churg-Strauss. Objective: This study is aimed to investigate the main demographic and clinical characteristics as well as the outcome of patients with systemic autoimmune diseases treated with TPE at an university hospital. PDN: prednison DIAGNOSIS MEAN AGE. Soyuoz A. 1(11%)without therapy. Results: A total of 60 patients (Female/male: 43/17) were treated with TPE. Patients with pulmonary and renal indications had increased mortality compared to patients with hematologic and neurologic indications. Conclusion: TPE is an effective therapeutic option for treating serious manifestations of systemic autoimmune diseases with rare adverse events. More than half of the patients with pulmonary (57. Helvaci O. The median application regimen of TPE was 6 (range 2-33) sessions with alternate days.(19-39 years) n¼22(81.. One patient died periprocedurely due to atrial fibrillation and myocardial infarction. The major adverse events were seen in 14 (23.Foetal-Maternal complication in 62. DMARD’s(44%). 1 catheter infection. 4 patients had dermato/polimyositis.2PA. Ozcebe O. NSAIDs: Nonsteroidal Antinflammatory Drugs.3% of patients TPE had been performed more than 10 times. UCTD: Undifferentiated Connective Tissue Disease. hepatic (5. NSAID(10%).0%).. fertility. SLE: Systemic Lupus Erithematosus.016.7% and 18. In 9 (15%) of patients TPE had performed in case of concomitant leucopenia or infection. Mean age at the time of 41.1. 1(11%)Ovarian Hyperstimulation Syndrome.-Pregnancies prophylaxis: 5(56%) LMWHþLDA. 38. 35. 2014 . Aim:To describe a case series of patients with fertility problems evaluated in our Clinic for Pregnancy in the Rheumatic Diseases. PA: Psoriasic Arthritis. 3 patients had primary antiphospholipide syndrome. 3 of them after discharge from hospital).13isolated Ab positivity.5UCTD. pulmonary (20. 12 patients with other diagnoses. [MEDIAN]. neurologic (28.3%) of patients died (17 of them during first treatment course. Italy Introduction: Infertility is an important topic for Rheumatologists because autoimmune diseases(AD) and related drugs can affect fertility of patients. Material and Methods: Patients treated with TPE between 2002-2013 were included into the study. DMARD’s(20%).72%hidiopatic secondary:2(7%) n¼10(37%): LDA(80%). complications and outcomes were obtained from hospital records. Totally 20 (33.(29-40years) n¼8(89%): Single:7(87%) Multiple:1(13%) primary:8(89%).Rheumathology and Clinical Immunology.1PAPS. pregnancy and neonatal care A097 INFERTILITY AND ASSISTED REPRODUCTIVE TECHNOLOGIES: OUR EXPIRIENCE IN AUTOIMMUNE DISEASES Reggia R.Moreover.2SjS. In 32. Kilic L.5 (range: 15-73 years). TPE could be a valuable option for those with refractory disease and/or concomitant infection. Twentyone (35.1 previous aPLpositivity. 1(11%) LMWH. LDA: Low Dose Aspirin.[38].. 6 patients had Sjogren syndrome. Iodice A.3 1 Johns Hopkins University School of Medicine. Gladman DD. Lojacono A. 1foetal-cardiac-malformation. Pregnant women with SLE were matched 1:5 with pregnant women without SLE. miscarriage.neurological manifestations(periodic syndromes and seizures and headache)and learning difficulties(in the nonverbal domain of functioning). all at term)in which coexist mild behavior disorders(Attention Deficit Hyperactivity Disorder and mood disorders). 1oligoidramnios. 2Lupus Foundation of America.4 Spedali Civili. Conclusion: Analysis of this large healthcare claims database shows that healthcare of pregnant women with SLE is associated with higher costs both during pregnancy and at delivery. Results: In all children the neurological physical exam was normal. Washington. and non-pregnant women with SLE. Andreoli L. making it difficult to determine the role of autoimmunity in infertility.1.2008. Belgium Introduction: Physician communication with women with SLE considering a family is critical for improving their care and support. Fazzi E. however our analyses revealed an unexplained reduction in these visits. References 1 Clowse et al Arthritis Rheum 2013 65(Suppl 10): S815.Neuropsychiatric aid in children born to patients with rheumatic diseases.2PROM(Premature Rupture Of Membranes).9th European Lupus Meeting 490 Restriction).Nacinovich R.com at Institute of Marine Biology of Crete (IMBC) on October 26.Department of Clinical and Experimental Sciences.Italy. Reflecting these differences.2 Spedali Civili and University of Brescia. USA. Brussels. Aim: To compare resource utilization in pregnant women with SLE to that in pregnant women without SLE and non-pregnant women with SLE. Patient and Methods: Review of records for women aged 12-54 years using retrospective analysis of a US healthcare claims database from 2006–2012. Conditional logistic regression was used to calculate p-values.28(5):767-73. Results: In comparison to pregnant women without SLE.Neurocognitive abnormalities in offspring of mothers with systemic Lupus Erythematosus. 2 Petri et al Arthritis Rheum 2013 65(Suppl 10):S1082.1.Intellectual functioning were performed with Wechsler scale for corrected age. As active disease is associated with higher rates of pregnancy complications regular interaction with the SLE specialist should be encouraged to ensure optimal management of both disease and pregnancy.Rheumatology and Clinical Immunology.All children showed a normal intelligence level. We submitted some questionnaires to the APS/SLE mothers: Child Behavior CheckList (CBCL)and an anamnestic home-made set of questions obtained by a multidisciplinary team(rheumatologists and pediatric neurologists)focused in particular on maternal drugs during pregnancy. Lupus Downloaded from lup.Motta M. USA. Aim: To evaluate the neurodevelopment outcome in 33 children (median age 6 years-old) born to mothers with SLE or APS with IgGantiBeta2Glycoprotein I (aB2GPI) positivity during pregnancy. in order to ensure optimal outcomes.Neonatology and NICU.Rovet J. pregnancy and puerperium seems to prevent thrombotic complications.Cerebelli A.Galli J.Fazzi E. 1preeclampsia.Iban˜ez D. Experts recommend more frequent rheumatologic visits during pregnancy for women with SLE. Medication use and frequency of clinic visits was also significantly different between pregnant women with SLE and non-pregnant women with SLE (Table 2). while it is understood that pregnant women with SLE have higher rates of pregnancy complications.The long-term outcome and the neurodevelopmental outcome of children born to patients with SLE and APS has been a matter of interest but only few data are available. 3UCB Pharma.Clin Exp Rheumatol. A098 LONG TERM OUTCOME IN CHILDREN BORN TO MOTHERS WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND ANTIPHOSPHOLIPID SYNDROME Nalli C. and Pushparajah D. We found a particular profile in 6 of these children(18%.Italy. Daly P.Lojacono A.2 their use of medications and hospital resources has not been extensively studied. baby developmental milestones.Brescia.MacKinnon A.Italy. The results are overall reassuring on a normal intelligence. SLE patients were isolated and pregnancies identified by including codes for live birth. scholastic performances. 2 Urowitz MB.2. while some minor disorders have been detected.. A099 RESOURCE USE IN PREGNANT WOMEN WITH SYSTEMIC LUPUS ERYTHEMATOSUS Petri M.Brescia.Brescia. Conclusions: This is the first study evaluating neurological manifestations in children born to aPL positive mothers with APS/SLE. These preliminary data must be extended and compared with matched controls for sex and age. gestational age. 2014 . Italy Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease that primarily affects women in childbearing age. References 1 Bomba M. Baltimore. Patients and Methods: A neurological physical exam was performed in all children.1.S.Obstetrics and Gynecology. healthcare costs were higher for pregnant women with SLE compared to pregnant women without SLE and nonpregnant women with SLE (Tables 1-2). pregnant women with SLE spent a significantly higher number of days in the maternity ward and required significantly more monitoring (Table 1). Motta M.sagepub.2 and Tincani A.4.Brescia.Department of Clinical and Experimental Sciences. This is predominantly due to increased fetal monitoring.-Maternal-foetal-complications occurred in more than half of the pregnancies. Conclusions: Most of the patients had additional risk factors and thyroid abnormalities. Antiphospholipid Syndrome (APS) is a systemic autoimmune disorder defined by the occurrence of thrombosis and pregnancy morbidity in presence of Antiphospholipid Antibodies(aPL).2.3 Spedali Civili and University of Brescia.Department of Clinical and Experimental Sciences. Careful pharmacological prophylaxis during hormonal stimulation. even if the risk of such cases is notoriously increased in gestations induced by ART. Pregnancies induced by ART are often treated with a ‘‘generous’’ prophylaxis.Silverman E.3. All children were IgG-aB2GPI positive at birth.Tincani A.1 However. even in the absence of risk factors.1 1 Spedali Civili and University of Brescia.Bruto V.2010 Sep-Oct. and ultrasound. Lupus:17(6):555-60.Unit of Child and Adolescent Neuropsychiatry. so its association with pregnancy is not a rare event. *p<C.4 years. All cases were managed with adjustment of medication.0% 19.6 6. A multidisciplinary approach with a pre-conception obstetric consultation is essential to prepare these patients pregnancy.4* 5.481 ja] Average number of vIsHsflests d urlng the to week analysis period.7* 1.9% 53. of which 60 % were iatrogenic preterm deliveries.1.8 3.7 % had clinical or analytical worsening during pregnancy and 6. 1Obstetric Department.4* 1. Patients and Methods: Retrospective descriptive study of all SLE pregnancies supervised by the same team at Autoimmune Disorders and Pregnancy Consultation of Oporto Hospital Center.8% 7. Regular specialized monitoring permits a early diagnosis of SLE exacerbations or other obstetric complications and allow the institution of adequate therapeutic options to assure the most favorable obstetric outcome.com at Institute of Marine Biology of Crete (IMBC) on October 26.1% 10. Vasconcelos C.8% 50. There were no cases of fetal death. Abbfrtlntfons NSAIDs.8* $21. Portugal Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease that affects women in childbearing age. QP. 22. Oporto.QQQ1 compared to Pregnant women without SLE.509* 0. major fetal malformations or severe neonatal morbidity.2 and Braga J. so multidisciplinary approach and specialized obstetric care are essential to assure the best outcome.4 % of pregnancies were complicated with fetal growth restriction. 95 % of cases continued the usual medication during pregnancy.4 1. Conclusions: Pregnant SLE patients are a group of high risk pregnancies. Lupus Downloaded from lup. jb] During the 44 week analysis period. 2014 .9th European Lupus Meeting 491 Table 1.591 [a] Average nu in ber of visit* d urlng the 44 week analysis period. Table 2. nonsteroidal anti-Inflammatory drugs. These pregnancies are full of challenges.1 1.4% (60 % with severity criteria).9%* 39.6 0. from January 2010 to September 2013.2 2. general practitioner A100 SYSTEMIC LUPUS ERYTHEMATOSUS AND PREGNANCY – OUR LAST 4 YEARS Braga A. Hydroxychloroquine was the most widely used drug (64% of cases).9 %. There was 1 case of neonatal lupus.7%* 42.8%* 24. 45 % were primiparous and 25 % had a history of miscarriage. 43% had SLE associated with other autoimmune diseases (15 % with antiphospholipid syndrome) and 15 % had renal involvement.2* $20. Results: 44 pregnancies were included in 42 patients with a mean age of 31.sagepub. The percentage of cesarean delivery was 40.9* 9.7* 1.8 6.1 $12.665* 5. There was an incidence of pre-eclampsia of 11. Internal Medicine Department. jb] During the 44 week analysis period.7%* 3.8 % postpartum.4% of cases had a preterm delivery.0* 2. *p<G. Resource use for pregnant women with SLE compared to pregnant women without SLE Resource Use [a] Fetal echocardiographies CVS/amnlocentesIs Fetal stress and non-stress tests Fetal ultrasounds OBQYN visits Days In the maternity ward Mean Total All-Cause Direct Healthcare Cost* [b] Pregnant Women with SLE (N¼1634) Pregnant women without SLE (N¼8605) 0.7 2.1 Oporto Hospital Center.CCC1 compared to non-pregnant women with SLE. 11.8 $11.6 2. Aim: To analyze the clinical characteristics and outcomes of SLE pregnancies in our institution. Medication and resource use for pregnant women with SLE compared to non-pregnant women with SLE Medication Use NSAIDs Methotrexate Mycophenolate Corticosteroids Hydroxychloroquine Resource Use [a] GP visits Outpatient hospital visits Rheumatologlst visits Internal medicine care provider visits Mean Total All-Cause Direct Healthcare Costs [b] Pregnant Women with SLE (N¼1634) Non-pregnant Women with SLE (N¼8170) 28. 11.5* 8. 2Clinical Immunology Unit.9%* 1. neonatal asphyxia. They answered a simple questionnaire. Material and methods: We evaluated 50 SLE female patients. but also to quantify what they really know about this problems. Hoellinger P.1..1 1 Rheumatology and 2Physical Medicine Departments. A submaximal multistage cycle ergometer test was performed at baseline and at week 12 to measure the physical working capacity at 75% of the predicted maximal heart rate per kg of body weight (PWC75%/kg) as an index of cardiorespiratory endurance (fitness index).2.2. NJ.com at Institute of Marine Biology of Crete (IMBC) on October 26. non-renal¼10). Conclusion: SLE has a huge impact both on women physiology. defined by a Krupp’s Fatigue Severity Scale (FSS) >3. Belgium Objective: Chronic fatigue (CS) is a very common symptom in patients suffering from systemic lupus erythematosus (SLE). SLE manifestations. median duration of follow –up was 36 months (6-50 months) prior to combo treatment and 21 months (6-60 months) after receiving the combo treatment. according to their place of residence. MD Background: Systemic lupus erythematosus (SLE) is often complicated by resistance or partial response to initial immunosuppressive regimen and flares after initial response. even when the disease is fully under control. A103 EFFECT OF TWO DIFFERENT EXERCISE PROGRAMS ON CHRONIC FATIGUE IN SLE PATIENTS Avaux M. Depresseux G. these being reflected on their reproductive status and perception.. With a much shorter fertile period. rest of the fertile patients do not desire to have a child in the near future. Results: A total of 43 SLE pts. Opris D. 3NIDDK. frequency of gynaecologic check-up. compliance to the training program was similar (48 and 54% of the expected hours in the HT and ST group. Hamilton. There was no difference in overall survival between CYC only and CYC and RTX combo groups. Stella Dali 2.3. not did patient’s perception of exertion during the cycle Lupus Downloaded from lup. SLE duration. 2014 . There was no significant increase in infections after receiving combo therapy. The PWC75%/kg did not change over time in none of the two groups.. with adequate follow up were identified: 31 (72%) received CYC.1. methods of contraception. Indications for CYC and RTX combination treatment was lupus nephritis(10/12). After being diagnosed with SLE. Waldman M. Except one pregnant patient.2. respectively). We present our experience with intravenous (IV) CYC plus RTX combination and its comparison to CYC only therapy. most commonly for recurrent flares of lupus nephritis. NIH..1 and Houssiau F. Three quarter of the patients is in menopause. MD. Patient’s demographic information. 12 (28%) received CYC plus RTX.1. concerning their disease or child status. Our study has inherent limitations due to its retrospective nature and small sample size.7. non-renal flares¼12) as compared to after receiving combo¼13(Renal¼3. Predetermined definitions (based on published literature) were used to record flares (renal vs. affecting especially women in the child-bearing age.9th European Lupus Meeting 492 A101 WOMEN HEALTH ISSUES IN A ROMANIAN SYSTEMIC LUPUS ERHYTEMATOSUS COHORT Saulescu I. The impact of the disease will be on the fertility. It has been suggested that this condition can be improved by exercice. usually after referral from other M. Main characteristics of this cohort are menopause usually before 45 years old and fearing of pregnancy. Only 6 patients from 25 being fertile are older then 40 years. Interestingly. In this 12-week study. Nieuwland-Husson S. Conclusion: CYC and RTX combination regimen may have efficacy in reducing renal and non-renal lupus flares. Bethesda. In the 12 patients who received CYC plus RTX. presence or absence of menopause. Patient’s perception of exertion during this procedure was evaluated by Borg’s scale. They associate pregnancy with a big risk. This does not appear to be associated with increased risk of infections. like osteoporosis or coronary heart disease. 64% of them being in early or premature menopause. There is limited data suggesting that the combination of cyclophosphamide (CYC) and rituximab (RTX) may have efficacy in recurrent and treatment resistant lupus. The program (3 times one hour/week) consisted in both groups of cardiotraining exercises (up the 60% of the maximal heart rate). partial or no response). Maria Hospital Background: SLE is the prototype of autoimmune disease. Methods: Data from patients who were treated with IV CYC monotherapy only or IV CYC and RTX combination from 2000-2013 was retrospectively reviewed. response to therapy and post-treatment infections were recorded. Bethesda. Results: While the FFS did not change at Week 12 in the control group. followed by muscle strengthening and stretching. Rizvi B. Groseanu L. Discussions about post-menopausal co-morbities and preventions are also mandatory in order to improve their quality of life. but also psychology.D. Combination regimen was prescribed based on clinician’s discretion for recalcitrant SLE. non-renal) and response (complete.1 1 NIAMS. it seems to be very important for them to be counselled on pros and cons pregnancy in every single case. patient-oriented care A102 CYCLOPHOSPHAMIDE AND RITUXIMAB COMBINATION TREATMENT FOR SEVERE LUPUS IS EFFECTIVE AND WELL TOLERATED Shahzad A. Cristina Pamfil Multidisciplinary. a significant improvement was noticed in both HT and ST groups. number of pregnancies and influence of SLE on family planning. number of flares before receiving CYC and RTX combo was significantly higher¼ 38(Renal flares¼26. Objective: to asses women health issues in SLE female patients and find out the impact of the disease on their reproductive and hormonal status.2. Nine additional SLE patients suffering from CF who did not trained were similarly tested and served as a control group. Results: Less then half go to a regular gynaecologic check-up. were randomly assigned the HT (n¼18) or ST (n¼15) group. Most used contraceptive measures are local barriers. just 4 patients accepted to continue their pregnancy. Cliniques Universitaires Saint-Luc.3 and Hasni S. Universite´ catholique de Louvain. Vasile R. but also will be related to menopause: early menopause will contribute to accrual damage. UMF Carol Davila Sf. The primary endpoint was the FSS at week 12. Before that all patients gave informed consent to process the information.sagepub. They were asked about period characteristics. Constantinescu C..A. Zhao X. In patients receiving combo treatment. Jaffri F. 42 consecutive patients with inactive SLE suffering from CF. relevant labs. Fraselle V. CNS lupus(1/12) and treatment resistant SLE(1/12). and Ionescu R. 2R-Research. Methods: After signed informed consent. Borangiu A. co-morbidities. Acknowledgments: 1. NIH. we compared the effects of a home training program without direct supervision (HT) and a physiotherapist-supervised training (ST). 1 and Fanouriakis A.sagepub. s. fatigue).014). these instructions need to be adjusted to the local culture. self-management is considered a critical element of care. Centro Hospitalar do Porto/UMIB/ICBAS/UP.1. s. FORTH.2. Martins Silva Ana A.2.d. Auditory Verbal Learning Test.965. aspects of information-giving (including timing and extent of information about prognosis and long-term risks). Marinho A. in opposition to its known favorable profile in others clinical manifestations. challenging health practitioners to provide patients with complete and adequate education and guidance. and Trail Making Test) was applied to 140 patients with SLE (97. Sidiropoulos P. References 1 Clin Rev Allergy Immunol.. Results and Discussion: The survey is in progress and results will be presented during the meeting. Farinha F.1. This logistic regression analysis also revealed that longer disease duration (adj. HCQ appears to reduce the risk of cognitive impairment in SLE. Almeida I.002). In order to provide instructions.9. Triantafillia K.4%.004). Corsi Test. Sentence Repetition. 33. and neuropsychiatric involvement). Acknowledgements: The study was supported by a grant of the Association Lupus Erythemateux (after a selection procedure performed by the Fonds pour la Recherche Scientifique en Rhumatologie/ Fonds voor Wetenschappelijke Onderzoek in de Reumatologie) p¼0.036) even when controlling for other covariates (i.614).7. The frequency of cognitive impairment was lower for patients currently taking HCQ (12. age. 2012. No significant association was found with age (p¼0. 2014 . 2 Arthritis Foundation of Crete.7. p¼0. Moreover. Patients and methods: SLE was diagnosed according to ACR criteria and patients taking HCQ had dosages between 100mg and 400mg/day.937. Digit Span.018) and neuropsychiatric involvement (adj. thereby indicating that tolerance to exercise was improved in compliant patients.1. Complex Figure Test. 2Unidade de Imunologia Clı´nica. the HT and the ST achieved similar positive results.1. education.42:145-53.012-1. Conclusions: In addition to preventing lupus relapses and having antithrombotic effects (1). p¼0. Ploumidou N. psychological and socioeconomic issues. and education coefficients of healthy subjects) was used as cut-off of deficit on 18 neuropsychological measures. 31.e.3.010) remained statistically associated to cognitive involvement.1 years. Aim: To explore the association between HCQ and cognitive impairment in SLE.001). Greece. Greece Background: As a result of rising medical costs and decreasing financial resources.2. There is a paucity of such data in patients living in Southern Europe.5. in the subgroup of HT and ST patients who achieved a 50% observance rate with the program.¼13. In this context. Gergianaki I.1 1 Rheumatology. Bertsias G. Patients with >3 neuropsychological measures with deficit were considered to have cognitive impairment. mean age at onset¼29. Santos E. odds ratio¼1. Results: Cognitive impairment (19.¼12. although this was not associated to improvement of the cardiorespiratory endurance at 3 months. 36. Conclusion: This study confirms the beneficial effects of exercise on CF in SLE patients. A comprehensive neuropsychological battery (Nine-hole Peg Test. The 5th percentile of regression based adjusted norms (based on sex. s. s. Issues addressed in the questionnaire include: patients’ beliefs regarding the causes of lupus and the factors that influence the disease. mean education¼10. A105 A104 HYDROXYCHLOROQUINE AND COGNITION IN SYSTEMIC LUPUS ERYTHEMATOSUS Cavaco S. Greece. Chi-square. nursing instructions are a cornerstone in influencing patient perceptions and facilitating self-care. 3 Institute of Molecular Biology-Biotechnology. unmet needs related to physical (pain. which needs to be supported by a permanent educative procedure. mean disease duration¼11.2 1 Servic¸o de Neurologia.368. Portugal Introduction: Available information on risk of cognitive impairment in systemic lupus erythematosus (SLE) is limited and the possible effects of hydroxychloroquine (HCQ) have not been explored yet. Experienced neuropsychologists blinded to the patients’ clinical records conducted the neuropsychological testing.¼7. mean age¼40. Lupus Downloaded from lup. Koutsogianni E. University of Crete. Melissourgaki M.338-8. odds ratio¼3. Contrary to our working hypothesis.1.3%) was significantly associated with years of education (p¼0.¼5. Porto. disease duration (p¼0.137. Interestingly.2. Moreira Ineˆs M. Wisconsin Card Sorting Test. MannWhitney test. nurses have to understand patient’s needs and factors affecting those needs.3. Clinical Immunology and Allergy. 95%CI: 1.2. Faria R.com at Institute of Marine Biology of Crete (IMBC) on October 26. as well as gaps in the collaboration of lupus physicians with primary care and specialty physicians. and multiple logistic regression were applied for data analyses. Aim: To better understand the needs and perspectives of lupus patients in a Southern European country.6% vs.1.d.1.2.144-0. Patients and Methods: A structured questionnaire has been developed in collaboration with the patients’ association (Arthritis Foundation of Crete). disease duration. p¼0.d.2. interest in self-management and telephone-based support. Starra A. Rapsomaniki P.5% vs.1. and neuropsychiatric involvement (11. probably because of a similar observance rates between the two groups. 95%CI: 0. Letter Word Fluency. The odds of having cognitive impairment was significantly lower for patients taking HCQ (adj.2. odds ratio¼0. In chronic diseases such as systemic lupus erythematosus.1.2.3%.9th European Lupus Meeting 493 ergometer test. Terizaki M.463.058) or age at onset (p¼0.4% with neuropsychiatric involvement) selected from the CHP’s Clinical Immunology Unit. hospital stays and outpatient visits have become shorter. Borg’s scale improved at week 12. Understanding lupus patients’ needs in the local cultural and socioeconomic context will enable nurses to interact more efficiently at the interface between physicians and patients and help the latter gain empowerment over their disease.2 and Vasconcelos C.d.1% women. Attentive Matrices.073. p¼0. Heraklion. A total of 50 patients were included in this survey. LUPUS PATIENTS’ NEEDS AND PERSPECTIVES IN SOUTHEASTERN EUROPE: DATA FROM THE CRETAN LUPUS COHORT ‘‘LETO’’ Kabouraki E.1.2. 95%CI: 1. in order to curve unnecessary delays before expert rheumatologic consultation and institution of appropriate therapy. United Kingdom. Fanouriakis A. Athens.001).02.1. Aberdeen. p < 0. variety of diagnostic tests ordered.3. 265) ¼ 9. University of Nottingham.05). Greece. 4 National and Kapodistrian University of Athens.02. Starra A.3. Bertsias G. family planning and employment was also assessed.04. King’s College London. p < 0. Ploumidou N. Heraklion. body pain (R2 ¼ 0. a country with a mixed health care system combining public and private facilities and services.03. p < 0. All patients underwent phone interview through a structured questionnaire.3 and Gergianaki I. 5th Department of Respiratory Diseases. 267) ¼ 7. SpringerVerlagLondon. London.4 1 General Hospital of Athens ‘‘G.5mg/kg/day).78.com at Institute of Marine Biology of Crete (IMBC) on October 26.1. References 1 Hughes GRVHughes Syndrome: A Patient’s Guide. Anastasopoulos A. Case description: A 31 year-old female patient was diagnosed with SLE a year ago based on the presence of malar rash. adjusted R2 ¼ 0. Migraine. Rheumatology Department. Data included social support (instrumental. vitality (R2 ¼ 0.04. United Kingdom. p < 0. 262) ¼ 12. F(1.1.J.44.2. F(1.2. United Kingdom Introduction: Antiphospholipid (Hughes) syndrome (APS) is characterized by recurrent venous and arterial thrombosis. memory loss. Lower instrumental support was a significant predictor of positive daily active (R2 ¼ 0. ANA titer of 1/320 and anti-SSA/ Ro antibodies were detected. Both lower instrumental and informational support provision significantly predicted increased physical limitations (R2 ¼ 0.1. University of Crete. adjusted R2 ¼ 0. 2Department of Psychology. The need for patienttailored interventions incorporating a bio-psychosocial approach is highlighted. Conclusions: Insufficient social support was predictive of limitations in various HRQOL domains in patients with APS.2. 6th Department of Respiratory Diseases. United Kingdom. 3 Institute of Molecular Biology-Biotechnology. Greece. Patients and methods: 270 APS patients with a clinical diagnosis of APS participated in a cross-sectional online questionnaire survey worldwide. Results: Multiple regression analysis indicated that specific types of social support were predictive of particular HRQOL domains.05.1. The impact of the disease on quality of life. Triantafillidou Ch. Neurological features of the antiphospholipid (Hughes’) syndromePostgrad Med J 2003. Medical School. 3Academic Urology Unit. After one year of follow-up the patient was still symptomatic with pleuritic chest pain and 3/4 in the MRC scale of dyspnea. Pleural effusion was exudative with lymphocytic predominance. Treatment with moderate/high steroid dose and immunosuppressives is effective in almost all cases of lupus pleuritis. and general health (R2 ¼ 0.1.2.3 1 Rheumatology. miscarriage. F(1.2. Miscellaneous . Terizaki M. The patient was treated with prednisolone (0. 2014 .1. Dimakou K.2. Athens.T. Greece. F(1.05). Athens. FORTH. neurological features such as stroke. A107 SOCIAL SUPPORT AND HEALTH-RELATED QUALITY OF LIFE IN PATIENTS WITH ANTIPHOSPHOLIPID (HUGHES) SYNDROME Georgopoulou S.2. and epilepsy1.4 1 Academic Department of Rheumatology. A complete workup for other causes of pleural effusion including infection and malignancy yielded negative results.sagepub.1. Triantafillia K. Aim: To investigate the relationship between social support and healthrelated quality of life (HRQOL) in patients with antiphospholipid (Hughes) Syndrome. Preliminary data suggest considerable delays along with significant unnecessary health care expenditures and a significant impact on quality of life and productivity. an unmet need exists in increasing public awareness around SLE. Patients and Methods: Patients (n¼50) with a first diagnosis of SLE within the last 3 years were recruited from an open call from the Arthritis Foundation of Crete. Athens. However pleural effusions recurred requiring repeated drainage and increased steroid doses with no evidence of improvement. adjusted R2 ¼ 0.60. memory loss.2. Greece. 2General Chest Diseases Hospital of Athens ‘‘Sotiria’’.02. adjusted R2 ¼ 0. F(1. Accordingly. and time to fist treatment after the onset of symptoms.1.03. number and specialty of physicians until the diagnosis of SLE was reached. Clinical Immunology and Allergy. Greece. F(1.34. adjusted R2 ¼ 0.001). Results and Discussion: This is an ongoing project and analysis will be completed at the time of the meeting. Birkbeck University of London.79. Aim: To analyze the use of health care services and map the course of patients from the time of first symptoms until the final diagnosis of SLE in the island of Crete.04.2 and Boumpas D.1 and Cox T. University of Aberdeen. Melissourgaki M. fatigue. Questions aimed to scrutinize the entire ‘‘journey’’ within the health care system and included: timing and type of first encounter with a health care service. Emotional support predicted positive mental health (R2 ¼ 0. Response rate was 60%. 2 Arthritis Foundation of Crete. arthritis and bilateral pleural effusion. Toumbis M. 2 Hughes G.Gennimatas’’.05. adjusted R2 ¼ 0. 79: 81–83. p < 0.06. normal glucose and high protein and LDH levels.04. Ibrahim F. 265) ¼ 6. p < 0.05). Greece Introduction: The course and usage of health care services of patients with a new diagnosis of systemic lupus erythematosus (SLE) has not been described in Greece. 261) ¼ 11.9th European Lupus Meeting 494 A106 MAPPING AND UTILIZATION OF HEALTH CARE SERVICES FROM PATIENTS WITH NEWLY DIAGNOSED SYSTEMIC LUPUS ERYTHEMATOSUS Rapsomaniki P. hydroxychloroquine 400mg/day and azathioprine 2mg/kg/day with resolution of her symptoms. Nottingham. Based on Lupus Downloaded from lup. Koutsogianni E. HRQOL (SF-36). MacLennan S. Little is known about the physical and psychological burden of this long-term condition.Difficult cases A108 A CASE OF SYSTEMIC LUPUS ERYTHEMATOSUS AND REFRACTORY BILATERAL PLEURAL EFFUSION AND REVIEW OF THE LITERATURE Karageorgas T. emotional and informational). demographics and disease-related information. photosensitivity.02. Kabouraki E. 4Centre for Sustainable Working Life. 3 General Chest Diseases Hospital of Athens ‘‘Sotiria’’. Efraimidou S. and ‘‘multiple sclerosis’’. These interventions would address psychosocial aspects of antiphospholipid (Hughes) Syndrome with the aim to improve patients’ psychological and physical status. 266) ¼ 6.001). headache. Greece Introduction: Pleuritis with or without pleural effusion is the most common respiratory manifestation in Systemic Lupus Erythematosus (SLE) occurring in approximately 45% of patients. and combined for all five reported cases to date ACR revised criteria for classification of systemic lupus erythematosus Patient 1 Combined for all 5 patients Patient 2 reported to date Malar rash Yes Yes 2/5(40%) Discoid rash No No 0/5 Photosensitivity Yes No 1/5(20%) Oral ulcers No No 1/5(20%) Non-erosive arthritis Yes Yes 5/5(100%) Pleuritis or Pericarditis Yes No 3/5(60%) Renal disorder No Yes 3/5(60%) Neurologic disorder No No 1/5(20%) Hematologic disorder Yes Yes 3/5(60%) Immunologic disorder Yes Yes 5/5(100%) Positive anti-nuclear antibody Yes Yes 4/4 tested (100%) Features of Myasthenia Gravis Patient 1 Patient 2 Combined for all 5 patients reported to date Generalised muscle weakness Yes Yes 5/5 (100%) Bulbar muscle weakness Yes No 2/5(40%) Anti-cholinesterase antibodies Yes Yes 5/5 (100%) Edrophonium challenge positive Yes Yes 4/4 tested (100%) Electromyogram positive Yes 3/3 tested (100%) Yes A110 CO-EXISTENCE OF JUVENILE-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS AND JUVENILE MYASTHENIA GRAVIS Sampath S. Birmingham. cyclophosphamide in 1/5 and hydroxychloroquine in 1/5. Department of Women’s and Children’s Health. 331(14–9. plasmapheresis in 3/5. only 5 cases being reported in literature at the moment. In case of refractory pleural effusion and severe multisystemic lupus activity treatment with high dose steroids and immunosuppressive therapy is usually employed.3. Systemic lupus erythematosus after thymectomy for myasthenia gravis: a case report and review of the literature. UMF Carol Davila Sf. Chylous ascites has rarely been described in patients with SLE. Introduction: Co-existence of Systemic Lupus Erythematous (SLE) and Myasthenia Gravis (MG) has been reported in adults (1. with a special focus on hydroxychloroquine use and a review of the literature. Liverpool. Beresford M.1 Alder Hey Children’s NHS Foundation Trust. Discussion: In children. et al. Spinty S. Lupus Downloaded from lup.1.1. Department of Paediatric Rheumatology 1 and Department of Paediatric Neurology 3. Discussion and Literature Review: Compared to the other cases described.9th European Lupus Meeting 495 the absence of SLE activity from other systems. the patient was referred for talc pleurodesis. being now on lamivudine treatment for almost 2 years. To increase awareness of the coexistence of these 2 rare conditions.1. Department of Paediatric and Adolescent Rheumatology.1. haematological.4 and McCann L. UK. Mean age at the time of diagnosis of JSLE and JMG were 10 (6 – 13) years and 12 (10 – 13) years respectively. She had in her history repeted abdominal surgical interventions before diagnose of SLE was established.4. Treatment for JSLE included glucocorticosteroids in all 5 patients. Journal of neurology 2012. 2014 . Eymard B. Cleary G. this patient presented recurrent ascites. Institute of Translational Medicine. Baildam E.sagepub. low complement levels consistent with chylous ascites in SLE patients. taking into consideration the evolution of the disease and the added pathology. Ethnicities of the 5 cases were: 2 Caucasian. However more data are needed on the long-term efficacy and complications of these measures. Here we have combined our two cases with 3 published cases to describe the clinical characteristics of these rare associations. High prevalence of systemic lupus erythematosus in 78 myasthenia gravis patients: a clinical and serologic study. Elbirt D. UK. association of JSLE and JMG is rare. In cases where pleural effusion (as in our case) represents the sole active manifestation of SLE. Table 1. Neiman A.1. Al Bshabshe A. intravenous immunoglobulin in 2/ 5 and thymectomy in 2/5. and developed viral hepatitis. symptoms of JMG preceded the symptoms of JSLE. et al. Case description: The laboratory analysis of the perithoneal fluid in our patient indicated high levels of triglycerides. Although diagnosis of JSLE preceded JMG in 3/5 cases. Jan.2. To the best References 1 Jallouli M. which presents within the serous involvement: recurrent chylous ascites. We recently diagnosed and treated 2 children with Juvenile-onset Systemic Lupus Erythematosus (JSLE) and Juvenile Myasthenia Gravis (JMG). positive antinuclear antibodies. 4/5 recalled episodic symptoms attributable to JMG prior to their JSLE symptoms.com at Institute of Marine Biology of Crete (IMBC) on October 26. we have collectively reviewed the clinical characteristics of 2 of our cases with 3 cases in the published literature. of our knowledge. immunological. All 5 patients had the generalised form of JMG at presentation. The American journal of the medical sciences 2006. Saadoun D. JSLE and JMG manifestations in these patients are described in Table 1. and Saulescu I. Liverpool.2 Birmingham Children’s Hospital NHS Foundation Trust.4. this one being apart from the reported ones. JMG was treated with pyridostigmine in all 5 patients. UK. 14(3272–6. The lab investigations of the fluid excluded malignancy (hematological or solid) or infections (including tuberculosis). 259(71290–7. topical treatment with pleurodesis and/or pleurectomy should be considered. there have been only 3 published case reports describing the co-existence of these two autoimmune diseases in children (3.2). A109 CHYLOUS ASCITES IN SYSTEMIC LUPUS ERHYTEMATOSUS – CASE PRESENTATION Vasile R. therapies achieved remission of JMG symptoms in 4/5. In the short-term. Discussion and Literature Review: Cases of refractory lupus pleural effusions are rarely reported in the literature.5). we will discuss the optimal course of treatment in this particular case. renal and serous manifestations. Maria Hospital. 2 Asian and 1 Arab. 2 Sthoeger Z. 4University of Liverpool. high number of leucocytes. Clinical and experimental nephrology 2010. Romania Intoduction and Aim: We describe a rare case of Systemic Lupus Erythematosus (SLE) with cutaneus. thus definite recommendations for the management of such cases is impossible to formulate. 3 Omar H. Alzahrani M. articular. laboratory findings and possible differential diagnosis for the chylous ascites. et al. In 4/5 cases. McDonagh J. Jul. The association of systemic lupus erythematosus and myasthenia gravis: a series of 17 cases. Clinical characteristics of two new patients. Bucharest. Pain C. mycophenolate mofetil in 2/5. Case description: All 5 patients were female. while she was under different courses of treatment. Apart from presenting the course of the disease. In addition to her chronic illness the patient was exposed to Hepatitic Virus B. . Tacrolimus was then administered to obtain a gradual improvement of proteinuria and systemic symptoms. he developed asteatotic eczema on his left leg with secondary infection and after several years his most delibating symptom was severe muscle spasm/cramps on his lower left leg. calcitriol. In March 2012. His blood count. Conclusions: Patients with morphoea may experience delay in diagnosis. Case report: In 1996 a 29-year-old male was referred to our dermatology department by his general practitioner with a 5 year history of sclerotic discoloured plaques on multiple sites and patchily on both legs with almost circumferential involvement of the left calf and ankle.9th European Lupus Meeting 496 4 Nishimura A. Jacobe H. DISABLING GENERALISED MORPHOEA ASSOCIATED WITH CALCINOSIS CUTIS: UNUSUAL CASE AND MANAGEMENT CHALLENGES Koumaki D. then was switched to mycophenolate mofetil and currently she is on low-dose steroids.C. Discussion: SLE is a multisystem autoimmune disease that affects various organs. Anti-nuclear antibodies. Department of Dermatology. Morphea (Localized scleroderma). 5 Rosskamp R. Pupo F. including the spleen. sodium. and Grattan C. Fuchigami T. University of East Anglia. creatinine. urea. Department of Internal Medicine. Histopathological examination of a skin biopsy confirmed the diagnosis of morphoea. Klin Padiatr 1983. Sometimes. Here we report an uncommon case of dystrophic calcinosis cutis associated with generalised morphoea that led to below knee amputation for arterial insufficiency and cramps. Because of the calcinosis cutis he could not wear a below-knee prosthesis and he was restricted to a wheelchair. In 2012 the disease was controlled with low dose steroids and an abdominal CT scan showed that multiple intraparenchimal splenic microcalcifications were unchanged. azathioprine and hydroxychloroquine. 149(91124. In this exceptionally unusual case the patient required amputation. Genoa. scleroderma. Antinuclear antibody (ANA) test was positive (1:640). or dermatomyositis. he was to have amputation of his left lower leg because of the cramps. with no improvement. when she presented with rash. 67(5881–889. Morphea in adults and children cohort II: patients with morphea experience delay in diagnosis and large variation in treatment. In the last years massive splenic microcalcifications with an unique pattern have been observed in few patients with SLE and it was reported they were not the consequence of comorbidities usually inducing splenic calcifications. rheumatoid factor and serologies for HIV and Borrelia were not reactive. JAMA Dermatol 2013 Sep. Localized linear scleroderma with cutaneous calcinosis. 195(6433–5. Movement in the latter was restricted. In 2010. but she had severe side effects (leukopenia and fever). University of Genoa. We speculate that in this patient spleen calcifications may represent an unusual immunological reaction of the spleen during SLE flares. and Bagnasco M.sagepub. Italy. Anti-phospholipid antibodies and lupus anticoagulant were negative. No To Hattatsu 1997. Yamazaki H.com at Institute of Marine Biology of Crete (IMBC) on October 26. DIFFUSE SPLEEN CALCIFICATION IN A PATIENT WITH SYSTEMIC LUPUS ERITHEMATOSUS (SLE) Rumbullaku M. Introduction: Spleen calcifications in SLE have been reported in a small number of patients. hydroxychloroquine. United Kingdom References 1 Fett NM. on examination there was calcinosis cutis on the shin of his left leg developed. ultrasound scan was performed and splenic microcalcifications were found.. Lupus Downloaded from lup. 2 Johnson W. in this examination. Nov-Dec. random blood sugar. his considerably atrophy of his left lower leg with abnormal skin with tension to erode and very poor circulation. calcinosis cutis could be a sequel of morphoea. and their significance in this disease is reason of debate.. C3 and C4 levels were reduced. anti dsDNA. fever. Puppo F. and anti-doublestranded (ds) DNA antibodies were present. During this time. ESR.. potassium and liver function tests were normal. In 2002. J Am Acad Dermatol 2012 Nov. Morita A. he experienced left below knee amputation. Sakakibara S. Abdominal ultrasound scans were performed and confirmed the presence of splenic microcalcifications. she had many flares with haematological and kidney involvement. 2014 . with a homogeneous pattern. Two years before this observation she had had an abdomen echography for abdominal pain and. the spleen was normal without any calcification. Pesce G. Cagnati P. In the meanwhile. A112 A111 Introduction: Dystrophic calcinosis is often noted in the subcutaneous tissues of connective tissues diseases–primarily systemic lupus erythematosus. et al. His general health was good. In August 2012. 3 Yamamoto A. 29(2112–114. 29(5390–5. Myasthenia gravis following lupus erythematosus disseminatus. Berdel D. J Dermatol 2002 Feb.E. Norfolk and Norwich University Hospital. Shintani Y. Case description: we report the case of a 34 year-old Caucasian woman in who SLE was diagnosed at the age of 13. In 2011 she had another disease flare with haematological and kidney involvement and was treated with cyclophosphamide and steroids. in conjunction with a flare.. Tsuji T. and intermittent arthritis.P. hematocrit. She was initially treated with steroids. The patient was started on oral prednisolone with penicillamine 250mg daily and with bath PUVA and for the next 15 he has tried several treatments such as penicillamine. Norwich. A childhood case of systemic lupus erythematosus associated with myasthenia gravis. which likely impacts outcome. Fiorucci M. with a unique pattern. 1.2. dermatology. Its clinical presentation as verrucous nodules or markedly hyperkeratotic plaques on the extensor surfaces of the extremities and/or the face.5. SLE.2 and Rednic S. Methods: A survey approach was used to appraise responses from students (N¼200) during preclinical studies (N¼100) and early in their clinical training (N¼100) in two medical schools in Greece (Universities of Athens and Crete).1. Pulse intravenous metilprednisolone and Cyclophosphamide along with Levosimendan (calcium sensitiser) were administered with resolution of the pulmonary infiltrates. Apostolopoulou A. to establish whether existing awareness of its challenging nature is sufficient to avert medical error. In 3 patients an erroneous clinical and pathological diagnosis was made prior to recognition of HCLE: 2 were diagnosed with squamous cell carcinomas in the perioral area resulting in inappropriate surgery. low C3 levels.1. Christofides A. Fanouriakis A. Because of the late recognition of lupus pneumonitis.9th European Lupus Meeting 497 A113 HYPERTROPHIC CUTANEOUS LUPUS ERYTHEMATOSUS: A DIAGNOSTIC PITFALL Walsh N. Aim: We studied our collective experience of this rare entity over the past decade. Halifax. bilateral lower limb edema. A115 LUPUS PNEUMONITIS WITH ACUTE RESPIRATORY DISTRESS SYNDROME AS INITIAL MANIFESTATION OF SLE Felea I.2.2. Ghib L. Sidiropoulos P. The hyperkeratotic nodules and plaques of HCLE were located on the face in 4 cases and on the extremities in 3. Rheumatology3. Results: Seven patients [2 men and 5 women] with HCLE were identified.3 Dermatopathology1. 2014 . In addition.1.1.sagepub. signs of heart failure due to acute myocarditis needing endotracheal intubation and mechanical ventilation. Laboratory tests revealed anemia. Discussion: ARDS caused by lupus pneumonitis is a rare entity and difficult to recognize. Linking the preclinical curriculum to clinical practice and exposing students to the multi. Case description: A 29 years old female patient was referred to our hospital with a diagnosis of sepsis 21 days after a cesarian section performed for late onset preeclampsia. Dermatology2. Bertsias G.J. among others. thereby leading to delayed medical intervention or inappropriate surgery. Banos A.1. In young patients with non. Lontos K. Biopsies were undertaken in 5 cases because of a clinical suspicion of malignancy and in 2 due to diagnostic uncertainty. Hagau N. Introduction: Hypertrophic cutaneous lupus erythematosus (HCLE). A diagnosis of SLE was made.1.T. 2 University of Crete Introduction: The transition from learning to the development of diagnostic and treatment competence remains a challenge in medical education. Immunological tests showed homogenous ANAs 1/2000. such as systemic lupus erythematosus (SLE) requires a multidisciplinary approach that embraces basic and clinical science concepts from various disciplines and may serve as an educational model system.1 and Johnson E. Aim: The objective of this study was to investigate the impression of students regarding the integration of basic and clinical science knowledge from various disciplines with regards to one complex disease. Green P.2. hypoproteinemia with hypoalbuminemia and nephrotic range proteinuria. cardiology.1. Andrei M. The patient was transferred to the ICU department. Conclusion: In this series from an academic medical centre there was a failure to recognize and appropriately treat HCLE in 3 of 7 patients upon initial presentation. A114 LUPUS IN MEDICAL EDUCATION: STUDENT AWARENESS OF BASIC. Antal O. the students’ perceptions about the etiology of lupus and factors that influence the course of the disease and management were also addressed. The diagnosis and treatment of disease processes with a complex pathophysiology.M. Upon admission she had dyspnea with 40 breaths/minute.2 and Hanly J. Patients and methods: Patients with HCLE were identified via clinical records in Rheumatology and Dermatology and cross referenced with those in Pathology. We present a case of lupus myocarditis and acute pneumonitis complicated with acute respiratory distress syndrome (ARDS) as the initial manifestation of SLE. positive ACL antibodies. high inflammatory markers. psychiatry. SaO2 98% with FiO2 0. Results and Discussion. Lung involvement usually appears later in the course of disease.com at Institute of Marine Biology of Crete (IMBC) on October 26. Aggressive treatment can lead to a favorable outcome. an uncommon variant of discoid lupus erythematosus (DLE). Canada.2. HCLE continues to elude diagnosis. clinical and pathological data were reviewed. Nova Scotia.1. is a challenging diagnosis. the other was diagnosed with psoriasis. The findings of this study will highlight the extent to which a multidisciplinary exposure to SLE that expands basic to clinical science enhances learning outcomes in medical education. The mean age was 49 years (range 30-61). Acute lupus pneumonitis is a rare manifestation with incidence raging from 1 to 12%. improvement of cardiac function and extubation after another 15 days. Frangou E. In 4 patients with systemic lupus erythematosus (SLE). The remaining 3 patients were managed by plastic surgeons. 3 had previously documented DLE and were under the care of a dermatologist and/or a rheumatologist. high titre anti DNAds antibodies.infectious alveolar infiltrates a diagnosis of lupus pneumonitis should be considered.2. mortality is high. Letsos A. CT exam raised the suspicion of ARDS or bilateral pneumonic processes. Cluj County Emergency Hospital. as well as basic knowledge in other relevant disciplines.1 1 Medical Schools National and Kapodistrian University of Athens. fever. and accompanying histopathological features simulate squamous cell carcinomas and actinic keratoses. elevated blood pressure (150/90mmHg). Boumpas D.and interdisciplinary aspects of medical care may enhance students understanding of the core components of disease processes. especially as a first manifestation of SLE. Pulmonary alveolar hemorrhage was excluded by bronho-alveolar lavage and cultures were negative. Her condition gradually worsened despite intravenous antibiotics and anticoagulation and 20 days after admission she developed respiratory failure. Leventis D. CLINICAL AND INTERDISCIPLINARY ASPECTS OF COMPLEX DISEASE PROCESSES Kerezoudis P. Cluj Napoca.1. A structured questionnaire assessed the students understanding of core concepts in basic immunology. Lupus Downloaded from lup.1 1 Rheumatology Department. Pulmonary X-rays showed bilateral basal infiltrates and massive pleural effusion. 2Anesthesia and Intensive Care Unit Department. Capital District Health Authority and Dalhousie University. nephrology. Romania Introduction: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with multiple organ involvement. Demographic. Despite cautionary evidence in the literature. including rheumatology.G. Abha. lymphadenopathy. Lupus Downloaded from lup. et al. KKU. For this reason. The blood tests showed hypochromic anemia and low ferritin. References 1 Xia JY. Germany Introduction and Aim: Treatment of systemic lupus erythematosus patients is sometimes challenging and requires an interdisciplinary approach. Rumbullaku M. Case description: We reported the case of a 57 years-old female suffering from undifferentiated connective tissue disease associated with anti-phospholipid antibodies syndrome. The patient is now asymptomatic. Cancer 1972. Case description: Here we report a 43 years old male patient who presented with progressive tetraparesis and right prefrontal cerebral changes in MRI in the neurology department. 2014 . central nervous system and others with apparent constitutional symptoms.F. arthralgias and few episodes of hemoptysis. Castleman’s disease (CD) is characterized by reactive lymphadenopathy with chronic inflammation. KKU. A case report of systemic lupus erythematosus combined with Castleman’s disease and literature review. Abha.. precordial tightness. 2King Khalid University. A few cases had been reported with Systemic Lupus Erythematosus. Italy Introduction: Pulmonary hemosiderosis is a rare condition characterized by recurrent episodes of diffuse alveolar hemorrhage that may lead to lung fibrosis. Lymphadenopathy is another common manifestation in active lupus and the cumulative incidence of at some point is high. Many organ can be affected in SLE like renal. Myasthenia gravis and Sjogren Syndrome. A117 SYSTEMIC LUPUS ERYTHEMATOSUS ASSOCIATED WITH CASTLEMAN’S DISEASES. and autoimmune or connective-tissue disease [2]. 4 Keller AR.. Computerized tomography demonstrated signs of emphysema rarefaction and bronchoscopy with BAL showed the presence of many hemosiderin-laden macrophages. Ernst-Moritz-Arndt-Universita¨t Greifswald. Since 2006 the patient reported low-grade fever. malignancy. so-called Devic syndrome. Pupo F. 29: 670–83.sagepub. Discussion: These findings lay a diagnose of pulmonary hemosiderosis related to autoimmune disease. Musculoskeletal Medicine J. It usually reveals with dyspnea. Patient: We present a case that fulfills the ACR criteria for SLE that responded to medical therapy but when flared with generalized lymphadenopathy proved to have CD.Faculty of Medicine. 32(72189–93) :. 9: 822–30. Castleman B.9th European Lupus Meeting 498 A116 PULMONARY HEMOSIDEROSIS AN UNUSUAL CASE Cagnati P. Puerantz A.Further diagnostic revelead an isolated CD4-lymphopenia. 2 Roberts J. The physical examination revealed and a tense abdomen and pain to small and medium-sized joints of the limbs. tetraparesis was solved and after remission he has been treated with belimumab. hemoptysis and secondary iron deficiency anemia. up to 60% [1]. Shalaby M. She had positivity for antinuclear (ANA) antibodies with a speckeld pattern (1:160). Iverson L. KSA Introduction: Systemic lupus erythematosus is an autoimmune disorder which affects mainly young adult females. Two months later she reported abdominal pain. Discussion: This case is illustrative due to the combination of several rare manifestations which offered several different therapeutic strategies. Menendez VP. Department of Internal Medicine.1 and Jamil A. Therefore MMF was discontinued. and Puppo F. In 2011 the patient withdrew HCQ because of ossesive-compulsive syndrome.After intensive interdisciplinary discussion with decided to treat this patient with cyclophosphamide intravenously in combination with steroids. Conclusion: The association of the CD with SLE is rare but because of the complexity of SLE and its broad differential diagnoses. Due to this lymphopenia initial immunosuppressive treatment was delayed. Xu F. KSA..[4] had been rarely associated with autoimmune connective tissue disease. Leo L. Hochholzer L. Fiorucci M. Then we treated the patient with MMF leading to clinical response. steroids and low dose acetyl salicylic acid. anti-cardiolipin and anti-b2-glycoprotein-1 antibodies as well as lupus–anticoagulant activity at high titers since 2006. 2012 Jul. anorexia. CASE PRESENTATION AND REVIEW OF LITERATURE. Colonscopy and scintigraphy with labeled blood cells resulted negative and gastroscopy revealed hiatal hernia in the absence of gastric mucosal lesions. including infection.Several differential diagnoses regarding those cerebral abnormalities were rouled out until a cerebral biopsy confirmed Lupus vasculitis.. Feb 8. In this poster we describe the diagnostic work-up and decision processes involving different medical specialties. arthralgias and several pericarditis episodes.A. Rheumatol Int . Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. [3] and manifests as angiofollicular lymph node hyperplasia. Medical Dept. A118 SYSTEMIC LUPUS ERYTHEMATOSUS – AN INTERDISCIPLINARY CHALLENGE Karnebeck V.C.The patient recovered. musckuloskeletal. 3 Castleman B. Bagnasco M. Genoa. antimalarials and vitamin D. 2012. those SLE patients who showed atypical presentation or with progressive adenopathy another overlooked underlying disease should be considered and one of them is Castleman’s disease.2 1 King Khalid University. Rheumatoid Arthritis. The classical therapy with high doses of corticosteroids was not possible in our case due to the patient’s psychiatric symptoms. The diagnosis is established by revealed hemosiderin-laden macrophages in broncho-alveolar lavage (BAL) or open lung biopsy. which has been reported to be an extreme rare disease. She started a therapy with hydroxychloroquine (HCQ) 200 mg. Later the patient was treated with azathioprine until 2009 when she showed segmental deep thrombophlebitis. Systemic Lupus Erythematosus with Features of Castleman Disease. Localized mediastinal lymph node hyperplasia resembling thymoma. University of Genoa. sometimes seen in autoinflammatory diseases.com at Institute of Marine Biology of Crete (IMBC) on October 26. Chen XY. she added to HCQ dicoumarol and mycophenolate mofetil (MMF). The differential diagnosis for diffuse lymphadenopathy is extensive.Faculty of Medicine Pathology Dept.Additionally positive aquaporin 4-antibodies could be detected which are seen in neuromyelitis optica. This combined therapy allowed remission of systemic manifestations and thrombophilia. striking interfollicular vascularity and dense interfollicular plasma cell type and/or eosinocytosis. Cancer 1956. and Fiene B. Observation: A 23 year-old woman without family story of SCD was admitted in department of internal medicine for anemia. These immune pathways may be deregulated in SLE patients. Ammouri W.4 g/l (2–4 g/l) and serum protein electrophoresis showed a polyclonal Ig G 24 g/l (range 9–13 g/l) with normal immunofixation. The occurrence of connectivite tissue diseases in particular systemic lupus erythematosus (SLE) has been rarely reported in SCD patients. the alopecia lesions were fluorescent and Microsporum canis was identified. Bachir H. Hepatitis C. Blood tests results showed normocytic anemia at 6. Harmouche H. all her hair grew in the next months. pain to the pressure of long bones and arthritis in knees. Blood cultures were negative. In this patient a probably chronic quiescent dermatophytosis was unmasked by the addition of a strong and new immunossupressive stimuli – rituximab. Abdominal ultrasonography. Rabat. 2014 . It remains to be understood what the real fungal infection role was on SLE global activity perpetuation before treatment. This report illustrates the importance of considering associated diseases when clinical findings are unexplained by SCD alone or are unresponsive to the conventional treatment. Thoraco-abdominal scan revealed numerous splenic infarctions. Due to similar clinical manifestations. On physical examination the patient was pale without jaundice. Early diagnosis and the initiation of appropriate treatment may decrease morbidity and mortality in these patients. The coombs test performed on admission and received later was strongly positive (IgG).com at Institute of Marine Biology of Crete (IMBC) on October 26. thransthoracic and transesophageal echocardiography were also normal. Maamar M. There is a slight splenomegaly.2. elevated lactodeshydrogenase (4670 UI/l) indirect hyperbilirubinemia (21 mg/l) with moderate cytolysis and cholestasis (ASAT 43 U/l. or the use of immunosupressive therapies. Immunological investigations revealed a positive anti-nuclear antibody (1/2600). After starting terbinafine.. arthralgia and fever. and Vasconcelos C. Discussion: The presence of infection in autoimmune patients poses a real challenge to the physician. Morocco Introduction: Sickle cell disease (SCD) is a prevalent genetic disorder that includes sickle cell anemia the homozygous and most common form of SCD (SS). Souissi.000/mcL. We report one patient who developed symptoms initially attributed to the SCD but in whom further investigations revealed an underlying SLE. ALAT 65U/l. she has not sickle cell crisis and SLE is actually quiescent. She responded poorly to steroids and didn’t respond to cyclosporine. Hepatitis B. PNN 9500/mm3) and moderate thrombopenia (100000/mm3). especially microbicidal and growth inhibitory activity of neutrophils. Symptoms had been developing for 6 weeks with alteration of general condition and abdominal pain.1. she presented with scalp vesicular lesions and rapidly progression to sepsis with periorbital and scalp cellulitis. Serology for human immunodeficiency virus. At 18 months follow up. severe hematological involvement (thrombocytopenia despite splenectomy and severe neutropenia due to oligoclonal T cell proliferation) and severe cutaneous involvement (patchy scalp alopecia with biopsy proven acute lupus). the diagnosis of SLE in patient with SCD may be difficult and often delayed. Discussion: The overlap of SLE and overt SCD is of interest but the limited number of patients that have been reported previously implies that the association is uncommon.2. Chest X ray examination was normal. she had a temperature of 39 5C. Cytobacteriologic urine analysis revealed no bacteria but microscopic hematuria (670/mm3) and leucocyturia (50/mm3). Centro Hospitalar do Porto.2 1 Universite´ Mohamed V. Faculte´ de Me´decine et de Pharmacie.2. brucellosis and typhoid fever were all negative. sickle cell hemoglobin C (SC) and sickle cell b thalassemia (S/b thal). Hemoglobin electrophoresis test showed Hb S at 50. Erythrocyt sediment rate was 110 mm first hour. Lupus Downloaded from lup. PAL217 U/l and gGT 188 U/l).sagepub. Instead. Sepsis resolved with large-spectrum antibiotics (carbapenem and vancomycin) and no bacterial agent was identified. Hb C at 44% and Hb A1 at 0% confirming the diagnostic of SCD (hemoglobin S/C). Rabat.1. On a black light lamp. Portugal Introduction: Systemic lupus erythematosus (SLE) patients have several mechanisms by which they are more susceptible to common and opportunistic infections. hyperleukocytosis with granulocytosis (leucocyt count 16500/mm3.3%. Maroc. either due to broad-based immune dysfunction . The neutropenia improved but the thrombocytopenia remains 50. tuberculin skin test was at 8 mm. the evolution was marked by the development of other arthritis in hands and relapse of anemia. Anti-phospholipid antibodies were negative. fibrinogen 6. Unidade de Imunologia Clı´nica. Proteinuria was negative. she is in clinical remission on prednisone 5 mg per day. The diagnosis of SLE associated to SCD was established with five criteria of American College of Rheumatology. blood pression was 130/75 mmHg and heart rate was 100/min. but ten days after the first infusion.1. Bone scan showed diffuse bone infarcts. Granulocyte colony-stimulating factor and rituximab were started.08 mg/l). C-reactive protein 38 mg/l (range <6 mg/l).1 and Adnaoui M.6 g/dl with high reticulocyts count (230000/mm3).1. Steroids were administered at a pulse of methyprednisolone 1g/day for 3 days followed by oral prednisone at 1 mg/kg/day with hydroxychloroquine.genetic or acquired due to disease itself (as dysfunction of B lymphocytes). Forty similar cases have been reported in the literature over the last 50 years. positive anti-Sm. Further investigations showed diminished haptoglobin (0. Immune responses to fungal antigens play an important role in the host defense against dermatophytosis. 2Internal Medicine departement. Case Description: Female 38 years old patient with longstanding SLE with pulmonary hypertension (on dual vasodilator therapy). A120 TINEA CAPITIS OVERINFECTION OF CUTANEOUS SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) UNCOVERED BY RITUXIMAB Alves R.9th European Lupus Meeting 499 A119 SYSTEMIC LUPUS ERYTHEMATOSUS IN A PATIENT WITH SICKLE CELL DISEASE Tazi Mezalek Z.1. Ibn Sina University Hospital. Faria R. bone pain. Her symptoms have quickly improved.
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