Revised International Chapel Hill Consensus Conference nomenclature of vasculitides

The Revised International Chapel Hill Consensus Conference nomenclature of vasculitides was formed by experts from 12 different countries in the field of vasculitis as a proposed update to the original 1994 International Chapel Hill Consensus Conference nomenclature of vasculitides. This 2012 update is the most widely used system for describing vasculitides. 

Classification

Primary vasculitides

• large vessel vasculitis: affecting predominantly large blood vessels (aorta and its major branches) (see large vessel vasculitides)

  • giant cell arteritis (temporal arteritis)

  • Takayasu arteritis

• medium vessel vasculitis: affecting predominantly medium blood vessels (main visceral arteries and their branches) 

  • polyarteritis nodosa

  • Kawasaki disease

• small vessel vasculitis: affecting predominantly small blood vessels (intraparenchymal arteries, arterioles, capillaries, and venules)

  • antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis

    • microscopic polyangiitis

    • eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome)

    • granulomatosis with polyangiitis (formerly known as Wegener granulomatosis)

  • immune complex small vessel vasculitis

    • anti-glomerular basement membrane (anti-GBM) disease (Goodpasture syndrome)

    • cryoglobulinaemic vasculitis

    • IgA vasculitis (Henoch-Schönlein purpura)

    • hypocomplementaemic urticarial vasculiti (anti-C1q vasculitis)

• variable vessel vasculitis: affecting no predominant type of vessel and can affect vessels of any size and type

  • Cogan syndrome

  • Behçet syndrome

• single-organ vasculitis: affecting vessels of any size in only a single organ

  • cutaneous leukocytoclastic angiitis

  • cutaneous arteritis

  • primary central nervous system vasculitis (see central nervous system vasculitides)

  • isolated aortitis (see aortitis)

  • others (e.g. idiopathic, pauci-immune pulmonary capillaritis)

Secondary vasculitides

• vasculitis associated with systemic disease

  • lupus vasculitis

  • rheumatoid vasculitis (see rheumatoid pulmonary vasculitis)

  • sarcoid vasculitis

  • others (e.g. Sneddon syndrome)

• vasculitis associated with probable etiology

  • hepatitis C virus-associated cryoglobulinaemic vasculitis

  • hepatitis B virus-associated vasculitis

  • syphilis-associated aortitis

  • drug-associated immune complex vasculitis

  • drug-associated ANCA-associated vasculitis (e.g. hydralazine, propylthiouracil, levamisole ^2,3) 

  • cancer-associated vasculitis

  • others (e.g. varicella zoster virus-associated vasculitis)

See also

• Lie classification of vasculitis

• pulmonary vasculitides