Systemic vasculitis syndrome | Common presenting features | Primary treatment |
Vasculitis of small vessels |
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Hypersensitivity vasculitis | Palpable purpura | Often self-limited if offending agent is removed. If isolated to skin, may not require therapy. In more severe cases, moderate- to high-dose corticosteroid therapy may be needed. |
Henoch-Schönlein purpura | Palpable purpura, arthritis, glomerulonephritis, intestinal ischemia | Often self-limited and requires no treatment. Steroid therapy for some cases of gastrointestinal or renal involvement. |
Cryoglobulinemia | Arthritis, Raynaud's phenomenon, glomerulonephritis, palpable purpura | Corticosteroids; plasmapheresis for severe involvement. Antiviral therapy required if associated with hepatitis C. |
Vasculitis of small and medium-sized vessels |
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Polyarteritis nodosa | Peripheral neuropathy, mononeuritis multiplex, intestinal ischemia, renal ischemia, testicular pain, livedo reticularis | High-dose corticosteroids, often with cytotoxic agents (e.g., cyclophosphamide [Cytoxan]) |
Microscopic polyangiitis | Pulmonary hemorrhage, glomerulonephritis | High-dose corticosteroids, often with cytotoxic agents (e.g., cyclophosphamide) |
Churg-Strauss vasculitis | Allergic rhinitis, asthma, eosinophilia, pulmonary infiltrates, coronary arteritis, intestinal ischemia | High-dose corticosteroids, often with cytotoxic agents (e.g., cyclophosphamide) |
Wegener's granulomatosis | Recurrent epistaxis or sinusitis, pulmonary infiltrates and/or nodules, glomerulonephritis, ocular involvement | High-dose corticosteroids and cyclophosphamide. Corticosteroids and methotrexate may be used for less severe involvement. |
Kawasaki disease | Fever, conjunctivitis, lymphadenopathy, desquamating rash, mucositis, arthritis, coronary artery aneurysms | High-dose aspirin and intravenous immune globulin |
Vasculitis of large vessels |
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Giant cell, or temporal, arteritis | Headache, polymyalgia rheumatica, jaw or tongue claudication, scalp tenderness, fever, vision disturbances | High-dose corticosteroids |
Takayasu's arteritis | Extremity claudication, athralgias, constitutional symptoms, renal ischemia | High-dose corticosteroids |
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