Table 1.

Names for vasculitides adopted by the 2012 International Chapel Hill Consensus Conference on the Nomenclature of Vasculitides. The items highlighted in red are changes or additions compared to the 1994 International Chapel Hill Consensus Conference on the Nomenclature of Vasculitides. Modified from reference 2.

Large vessel vasculitis (LVV)

Takayasu arteritis (TAK)

Giant cell arteritis (GCA)

Medium vessel vasculitis (MVV)

Polyarteritis nodosa (PAN)

Kawasaki disease (KD)

Small vessel vasculitis (SVV)

Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV)

Microscopic polyangiitis (MPA)

Granulomatosis with polyangiitis (Wegener’s) (GPA)

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA)

Immune complex SVV

Anti-glomerular basement membrane (anti-GBM) disease

Cryoglobulinemic vasculitis (CV)

IgA vasculitis (Henoch-Schönlein) (IgAV)

Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis)

Variable vessel vasculitis (VVV)

Behcet’s disease (BD)

Cogan’s syndrome (CS)

Single-organ vasculitis (SOV)

Cutaneous leukocytoclastic angiitis

Cutaneous arteritis

Primary central nervous system vasculitis Isolated aortitis

Others

Vasculitis associated with systemic disease

Lupus vasculitis

Rheumatoid vasculitis

Sarcoid vasculitis

Others

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

Others