Polymyalgia Rheumaticapitfalls in diagnosis PMR & GCA Closely linked conditions ? Different phases of the same disease PMR GCA there is In situ production of cytokines in the temporal arteries of patients with PMR who do not have histological evidence of arteritis This suggests the presence of a subclinical vasculitis in PMR . Clinical features of PMR Systemic: Fever Anorexia Malaise Weight loss May present as PUO . Proximal: Stiffness & pain in Shoulders Neck Hips Worse with activity Difficulty getting out of bed . Distal: Non-erosive arthritis (wrists & knees) CTS RS3PE . nodular.Clinical features of GCA Headache: temporal / occipital Scalp tenderness ? Thickened. tender TA ? / absent pulse Jaw claudication: internal maxillary artery Tingling tongue: lingual artery Amaurosis fugax Unilateral permanent loss of vision other eye affected within 1-2 wks (ophthalmic & posterior ciliary arteries) . o TA Bx .phos.q -anaemia of ´chronic diseaseµ Alk.Diagnosis: ESR o /CRPo .normal in 10% Hb. n Fragmentation of internal elastic lamina . o Inflammatory infiltrate . o giant cell . Diagnostic criteria of PMR Bird et. al: Age > 65 ESR > 40 Onset within 2 weeks Bilateral shoulder pain &/or stiffness Morning stiffness > 1 hour Bilateral upper arm tenderness Depression &/or Wt loss . Diagnosis requires Any 3 of 7: Sensitivity 92% Specificity 80% . Problems in applying diagnostic criteria 40% have symptoms>3 months before referral 20% delay in hospital diagnosis>1 month 10% PMR with normal ESR . Sensitivity 92% FN = 1 ² sensitivity = 8% Specificity 80% FP = 1 ² specificity = 20% . conditions frequently confused with polymyalgia? . Shoulder pain & stiffness Frozen shoulders Rheumatoid arthritis Fibromyalgia Myxoedema . Proximal weakness & tiredness Polymyositis Thyrotoxic myopathy Osteomalacia Carcinomatous myopathy . Raised ESR Myeloma Malignancy . followed up between 1995-1997 Haugeberg et.al .Prospective study of 50 patients in Norway with PMR symptoms. PMR TA collaginosis coxarthosis Shoulder tendinitis Prostate ca Liver mets unknown 1° myelodysplasia Lymphoma Total 40 2 1 1 1 1 1 2 1 50 Haugeberg.et. al . The observed frequency of malignancy in these patients was compared with the frequency of expected malignancy in the Norwegian population over the same period adjusted for age and sex. given by the national cancer registry . 6% Frequency in study population = 10% A two-tailed Fisher·s exact test: P = 0. Age & sex-adjusted frequency of malignancy in general population = 1.0013 . et. al .PMR TA collaginosis coxarthosis Shoulder tendinitis Prostate ca Liver mets unknown 1° myelodysplasia Lymphoma Total 40 2 1 1 1 1 1 2 1 50 Haugeberg. TA) = 8 = 16% Specificity = 80% FP = 20% .Total non (PMR. A tricky disease .PMR. May present as PUO May have normal ESR May mimic hidden malignancy EORA may present as PMR: Anti-CCP may differentiate GCA May present with different arterial syndromes . Features raising suspicion of other diagnoses: S Siebert et. al younger normal presenting age(< 65) inflammatory markers to low dose steroids raised ALP non-response persistently . Ca. if it does not investigate further . serum electrophoresis and dipstick urinalysis ALP should to normal after 3/52 treatment. CK. ESR. U&Es. LFTs TFT. Patients with suspected PMR should have baseline: FBC. CRP. Failure to respond to steroids should raise doubts about the diagnosis of PMR A good response to steroids does not necessarily confirm the diagnosis The presence of any atypical features should prompt more detailed investigation .