ICD-11 classes
04 Diseases of the immune system
Nonorgan specific systemic autoimmune disorders
4A44 — Vasculitis
4A44.A — Antineutrophil cytoplasmic antibody-associated vasculitis
4A44.A1 — Granulomatosis with polyangiitis

ICD-11 4A44.A1 — Granulomatosis with polyangiitis

Necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotizing vasculitis affecting predominantly small to medium-sized vessels (e.g., capillaries, venules, arterioles, arteries and veins). Necrotizing glomerulonephritis is common . (Arthritis Rheum 1990; 33:1101-1107)

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

• Antineutrophil cytoplasmic antibody-associated vasculitis #16612
• Antineutrophil cytoplasmic antibody-associated vasculitis #18414