2012 Chapel Hill Consensus Conference definitions for ANCA-associated vasculitis
| Definitions for ANCA-associated vasculitis | |
|---|---|
| ANCA-associated vasculitis (AAV) | Necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels (ie capillaries, venules, arterioles and small arteries), associated with MPO-ANCA or PR3-ANCA. Not all patients have ANCA. Add a prefix indicating ANCA reactivity, eg PR3-ANCA, MPO-ANCA, ANCA-negative. |
| Granulomatosis with polyangiitis (Wegener’s) | Necrotising granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotising vasculitis affecting predominantly small to medium vessels (eg capillaries, venules, arterioles, arteries and veins). Necrotising glomerulonephritis is common. |
| Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) | Eosinophil-rich and necrotising granulomatous inflammation often involving the respiratory tract, and necrotising vasculitis predominantly affecting small to medium vessels, and associated with asthma and eosinophilia. ANCA is more frequent when glomerulonephritis is present. |
| Microscopic polyangiitis | Necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels (ie capillaries, venules or arterioles). Necrotising arteritis involving small and medium arteries may be present. Necrotising glomerulonephritis is very common. Pulmonary capillaritis often occurs. Granulomatous inflammation is absent. |
ANCA = antineutrophil cytoplasmic antibody; MPO = myeloperoxidase; PR3 = proteinase 3
Adapted from Jennette et al.2