TABLE 1.

CLASSIFICATION OF THE VASCULITIDES

Primary idiopathic vasculitis

Small vessel

Wegener's granulomatosis

Churg-Strauss syndrome

Microscopic polyangiitis

Idiopathic pauci-immune rapidly progressive glomerulonephritis

Isolated pauci-immune pulmonary capillaritis

Medium vessel

Polyarteritis nodosa

Kawasaki disease

Large vessel

Giant cell arteritis

Takayasu's arteritis

Primary immune complex–mediated vasculitis

Goodpasture's syndrome

Henoch-Schönlein purpura

Behçet's disease

IgA nephropathy

Secondary vasculitis

Classic autoimmune disease

Systemic lupus erythematosus

Rheumatoid arthritis

Polymyositis/dermatomyositis

Scleroderma

Antiphospholipid antibody syndrome

Essential cryoglobulinemia

Inflammatory bowel disease

Hypocomplementemic urticarial vasculitis

Drug-induced (e.g., propylthiouracil, diphenylhydantoin)

Paraneoplastic

Infection

Among the primary vasculitides, those that affect the small vessels, Wegener's granulomatosis, Churg-Strauss syndrome, and microscopic polyangiitis, are grouped together as the ANCA-associated vasculitides. The vasculitides associated with pathologic granulomata include Wegener's granulomatosis, Churg-Strauss syndrome, Takayasu arteritis, and giant cell arteritis.