ICD-11 classes
04 Diseases of the immune system
Nonorgan specific systemic autoimmune disorders
4A44 — Vasculitis

ICD-11 4A44 — Vasculitis

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.

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code 4A44 contains 16 clarifying diagnoses:

1. 4A44.0 — Rhizomelic pseudopolyarthritis
2. 4A44.1 — Aortic arch syndrome
3. 4A44.2 — Giant cell arteritis
4. 4A44.3 — Single organ vasculitis
5. 4A44.4 — Polyarteritis nodosa
6. 4A44.5 — Mucocutaneous lymph node syndrome
7. 4A44.6 — Sneddon syndrome
8. 4A44.7 — Primary angiitis of the central nervous system
9. 4A44.8 — Thromboangiitis obliterans
10. 4A44.9 — Immune complex small vessel vasculitis
    It contains 6 clarifying diagnoses.
11. 4A44.A — Antineutrophil cytoplasmic antibody-associated vasculitis
    It contains 5 clarifying diagnoses.
12. 4A44.B — Leukocytoclastic vasculitis
    It contains 3 clarifying diagnoses.
13. 4A62 — Behçet disease
14. 3B65 — Thrombotic microangiopathy, not elsewhere classified
    It contains 2 clarifying diagnoses.
15. 4A44.Y — Other specified vasculitis
16. 4A44.Z — Vasculitis, unspecified

The diagnosis is coded elsewhere:

• Nonorgan specific systemic autoimmune disorders #18345
• Vasculitis #18397