Table 1.

CHCC 2012 categories of ANCA-associated vasculitis (modified from reference (1))

CHCC 2012 NameCHCC 2012 Definition
ANCA-associated vasculitisNecrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels (i.e., capillaries, venules, arterioles, and small arteries), associated with myeloperoxidase (MPO) ANCA or proteinase 3 (PR3) ANCA. Not all patients have ANCA. Add a prefix indicating ANCA reactivity, e.g., MPO-ANCA, PR3-ANCA, ANCA-negative.
Microscopic polyangiitisNecrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels (i.e., capillaries, venules, or arterioles). Necrotizing arteritis involving small and medium arteries may be present. Necrotizing GN is very common. Pulmonary capillaritis often occurs. Granulomatous inflammation is absent.
Granulomatosis with polyangiitis (Wegener)Necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotizing vasculitis affecting predominantly small-to-medium vessels (e.g., capillaries, venules, arterioles, arteries, and veins). Necrotizing GN is common.
Eosinophilic granulomatosis with polyangiitis (Churg–Strauss)Eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small-to-medium vessels, and associated with asthma and eosinophilia. ANCA is more frequent when GN is present.
  • CHCC 2012, 2012 International Chapel Hill Consensus Conference on the Nomenclature of Vasculitides.