4A44

ICD-11 MMS 2026-01

Vasculitis

Description

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.

Exclusions from above levels

  • 02 – Neoplasms
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 20 – Developmental anomalies

Child codes

  • 4A44.0 – Rhizomelic pseudopolyarthritis
  • 4A44.1 – Aortic arch syndrome
  • 4A44.2 – Giant cell arteritis
  • 4A44.3 – Single organ vasculitis
  • 4A44.4 – Polyarteritis nodosa
  • 4A44.5 – Mucocutaneous lymph node syndrome
  • 4A44.6 – Sneddon syndrome
  • 4A44.7 – Primary angiitis of the central nervous system
  • 4A44.8 – Thromboangiitis obliterans
  • 4A44.9 – Immune complex small vessel vasculitis
  • 4A44.A – Antineutrophil cytoplasmic antibody-associated vasculitis
  • 4A44.B – Leukocytoclastic vasculitis
  • 4A44.Y – Other specified vasculitis
  • 4A44.Z – Vasculitis, unspecified

Foundation children elsewhere

  • 3B65 – Thrombotic microangiopathy, not elsewhere classified
  • 4A62 – Behçet disease

Sibling codes

  • 4A40 – Lupus erythematosus
  • 4A41 – Idiopathic inflammatory myopathy
  • 4A42 – Systemic sclerosis
  • 4A43 – Overlap or undifferentiated nonorgan specific systemic autoimmune disease
  • 4A45 – Antiphospholipid syndrome
  • 4A4Y – Other specified nonorgan specific systemic autoimmune disorders
  • 4A4Z – Nonorgan specific systemic autoimmune disorders, unspecified

Foundation entity

Vasculitis – http://id.who.int/icd/entity/572581721

Allowed postcoordination axes

Part of: 04: Diseases of the immune system