1. Vasculitis and connective tissue disease – just a taster!! The common and the rare!!

2. Polymyalgia rheumatica (PMR) Common clinical syndrome of middle- aged & elderly (2-3 / GP) characterized by pain & stiffness neck, shoulder & pelvic girdles dramatic response to small doses of steroids

3. Giant cell arteritis vasculitis underlying polymyalgia rheumatica and temporal arteritis Other terms cranial arteritis & granulomatous arteritis Early recognition & treatment can prevent blindness & other complications resulting from occlusion or rupture of involved arteries

4. Polymyalgia rheumatica musculoskeletal symptoms usually bilateral & symmetric Stiffness predominant feature; particularly EMS may prevent patient getting out of bed Muscular pain often diffuse; pain at night common Corticosteroid treatment usually required for at least 2 years. Sometimes 4-5 years Systemic features include low-grade fever, fatigue, weight loss & an increased erythrocyte sedimentation rate Prednisolone 20 – 30mg / day ( tapering over 2 years or so)

5. Giant cell arteritis wide range of symptoms, clinical findings related to involved arteries Frequently fatigue, headaches, jaw claudication, loss of vision, scalp tenderness, AND polymyalgia rheumatica Risk is loss of vision – this is a medical emergency! Prednisolone 40 – 60 mg / day ( tapering over 2 years or so)

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8. Figure 157.2 Granulomatous vasculitis. (a) Brain biopsy findings of granulomatous vasculitis side by side with a polyarteritis-type necrotizing arteritis (open arrow). (b) Close-up view of (a) showing foreign body (short arrow) & foreign body (long arrow) giant cells in granulomatous vasculitis. Hematoxylin & eosin, × 64 & 400 respectively. (With permission from Calabrese et al. 4) Downloaded from: Rheumatology (on 18 November 2006 12:39 PM) © 2005 Elsevier

9. 'vasculitis' heterogeneous group of disorders characterized inflammatory alteration of blood vessel wall primary event (primary vasculitis) - e.g. in ANCA- associated vasculitides or secondary to other diseases (secondary vasculitis), e.g. in RA or SLE underlying events mediated by immunologic mechanisms supported by response to various potent immunosuppressives Aggressive treatment to prevent permanent organ damage

10. Few clues to aetiology of primary vasculitides Hep B virus implicated as causative agent in polyarteritis nodosa (PAN) & Hep C in essential mixed cryoglobulinemia (EMC) circumstantial evidence for causative role of infection in Wegener's granulomatosis: seasonal variation in incidence of proteinase 3 autoantibodies (PR3-ANCA) suggests that an infectious agent prevalent in spring or autumn might trigger WG genetic predisposition suggested by studies showing link between small vessel vasculitis & HLA-DQw7, between WG & DR1, & between HLA-Bw52 & giant cell arteritis

11. To name a few…….. Giant cell arteritis Takayasu arteritis Polyarteritis nodosa Kawasaki disease Wegeners Granulomatosis Churg-Strauss Syndrome Microscopic polyangiitis Henoch-Schonlein purpura Essential cryoglobulinaemic vasculitis Behcets disease Cutaneous leukocytoclastic vasculitis

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13. Figure 155.6 Leukocytoclastic vasculitis with ulceration in a patient with rheumatoid arthritis. Downloaded from: Rheumatology (on 19 November 2006 12:03 AM) © 2005 Elsevier

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16. Figure 146.4 Polyarteritis involving the gallbladder artery, showing pleomorphic inflammatory cell infiltration & fibrinoid necrosis. (With permission from Conn 10.) Downloaded from: Rheumatology (on 19 November 2006 12:03 AM) © 2005 Elsevier

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19. Figure 146.6 Vasculitis pathogenesis Downloaded from: Rheumatology (on 19 November 2006 12:03 AM) © 2005 Elsevier

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26. Connective tissue disease group of conditions which affect many organs or tissues widespread inflammation Have some symptoms in common All patients have features of general inflammation : fever, weight loss, tiredness & anaemia Most patients also have inflammation of joints Vasculitis of small blood vessels also common Raynaud’s phenomenon Involvement of the kidney may result in hypertension If inflammation settles all symptoms improve However, if process continues scarring occurs in affected tissues - will be damaged permanently

27. As well as symptoms common to whole group, each CTD has own particular symptoms All can be distinguished clinically & serologically All these CTD are rare All more common in women All treated with immunosuppressant drugs

28. SCLERODERMA; excessive fibrosis of tissues; skin is hard, & loses its ability to stretch. The fingertips are thickened & skin around the mouth tightens POLYMYOSITIS; inflammation mainly in muscles; pain & weakness are main symptoms. Lung fibrosis SLE; skin, joints, fatigue, mouth ulcers, alopecia, glomerulonephritis, lung fibrosis, CNS involvement Sjogrens; sicca symptoms, joints, fatigue, lymphoma risk Also may have symptoms from different disorders; MIXED CONNECTIVE TISSUE DISEASE

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36. Figure 122.6 Mouth ulcers in a patient with SLE. Downloaded from: Rheumatology (on 18 November 2006 12:39 PM) © 2005 Elsevier