Table 17.23-1. Nomenclature of vasculitides adopted by the 2012 International Chapel Hill Consensus Conference

Large-vessel vasculitis

Takayasu arteritis

Giant cell arteritis

Medium-vessel vasculitis

Polyarteritis nodosa

Kawasaki disease

Small-vessel vasculitis

ANCA-associated vasculitis:

– Microscopic polyangiitis

– Granulomatosis with polyangiitis (Wegener granulomatosis)

– Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Immune-complex small-vessel vasculitis:

– Anti–glomerular basement membrane (anti-GBM) disease

– Cryoglobulinemic vasculitis

– IgA vasculitis (Henoch-Schönlein purpura)

– Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)

Variable-vessel vasculitis

Behçet disease

Cogan syndrome

Single-organ vasculitis

Cutaneous leukocytoclastic angiitis

Cutaneous arteritis

Primary central nervous system vasculitis

Isolated aortitis

Other

Vasculitis associated with systemic disease

Lupus vasculitis

Rheumatoid vasculitis

Sarcoid vasculitis

Other

Vasculitis associated with probable etiology

Hepatitis C virus–associated cryoglobulinemic vasculitis

Hepatitis B virus–associated vasculitis

Syphilis-associated aortitis

Drug-associated immune-complex vasculitis

Drug-associated ANCA-associated vasculitis

Cancer-associated vasculitis

Other

Source: Arthritis Rheum. 2013;65(1):1-11.

ANCA, antineutrophil cytoplasmic antibody.