Table 1

Chapel Hill Consensus Conference definitions for ANCA-associated vasculitis

Disease Definition
ANCA-associated vasculitisNecrotising vasculitis, with few or no immune deposits. Small vessels (ie, capillaries, venules, arterioles and small arteries) are predominantly affected. Necrotising arteritis of small/medium arteries may accompany. It is associated with MPO-ANCA/PR3-ANCA, but ANCAs are not always found in all patients.
MPANecrotising vasculitis, with few or no immune deposits. Small vessels (ie, capillaries, venules, arterioles and small arteries) are predominantly affected. Necrotising arteritis of small/medium arteries may accompany. Necrotising glomerulonephritis is very common. Pulmonary capillaritis also often occurs. Granulomatous inflammation does not occur.
GPANecrotising granulomatous inflammation primarily affecting the upper and lower respiratory tract, and necrotising vasculitis predominantly affecting small and medium vessels (capillaries, venules, arterioles, arteries and veins). Necrotising glomerulonephritis is usually found.
  • ANCA, antineutrophil cytoplasm antibody; GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis; MPO, myeloperoxidase; PR3, proteinase3.