ICD-11

Vasculitides represent a heterogenous group of diseases of multifactorial aetiology characterised by inflammatory lesions of vessels. These lesions consist of fibrinoid necrosis (necrotizing arteritis), giant cell infiltration without necrosis, immunoglobulins deposit or leukocytoclastic infiltration. The spectrum and severity of the systemic vaculitides is broad, from life or sight threatening fulminant disease to relatively minor skin disease.

Necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels (i.e., capillaries, venules, arterioles and small arteries), associated with MPO-ANCA or PR3-ANCA. Not all patients have ANCA. Add a prefix indicating ANCA reactivity, e.g. PR3-ANCA, MPO-ANCA, ANCA-negative.

Eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium-sized vessels, and associated with asthma and eosinophilia. ANCA is most frequent when glomerulonephritis is present. (Arthritis Rheum 1990;33:1094-1100)