Systemic vasculitis syndrome	Common presenting features	Primary treatment
Vasculitis of small vessels
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
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
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