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. 2006 Mar;3(1):48–57. doi: 10.1513/pats.200511-120JH

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.