Revised International Chapel Hill Consensus Conference nomenclature of vasculitides
Following months of preparation, experts in the field of vasculitis from 12 different countries proposed an update to the 1994 International Chapel Hill Consensus Conference nomenclature of vasculitides. This 2012 update at the time of writing (mid-2016) remains the most widely used system for describing vasculitides.
Classification
Primary vasculitides
- large vessel vasculitis (LVV): affecting predominantly large blood vessels (aorta and its major branches) (see large vessel vasculitides)
- Takayasu arteritis (TAK)
- giant cell arteritis (GCA) (temporal arteritis)
- medium vessel vasculitis (MVV): affecting predominantly medium blood vessels (main visceral arteries and their branches)
- polyarteritis nodosa (PAN)
- Kawasaki disease (KD)
- small vessel vasculitis (SVV): affecting predominantly small blood vessels (intraparenchymal arteries, arterioles, capillaries, and venules)
- antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)
- microscopic polyangiitis (MPA)
- eosinophilic granulomatosis with polyangiitis (EGPA) (formerly known as Churg-Strauss syndrome)
- granulomatosis with polyangiitis (GPA) (formerly known as Wegener granulomatosis)
- immune complex SVV
- anti-glomerular basement membrane (anti-GBM) disease (Goodpasture syndrome)
- cryoglobulinaemic vasculitis (CV)
- IgA vasculitis (IgAV) (Henoch-Schönlein purpura)
- hypocomplementaemic urticarial vasculitis (HUV) (anti-C1q vasculitis)
- antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)
- variable vessel vasculitis (VVV): affecting no predominant type of vessel and can affect vessels of any size and type
- Cogan syndrome (CS)
- Behçet syndrome (BS)
- single-organ vasculitis (SOV): 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 (eg. 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. propylthiouracil )
- cancer-associated vasculitis
- others (e.g. varicella zoster virus-associated vasculitis)
See also
