4A44.3

ICD-11 MMS 2026-01

Single organ vasculitis

Description

Vasculitis in arteries or veins of any size in a single organ that has no features that indicate that it is a limited expression of a systemic vasculitis. The involved organ and vessel type should be included in the name (e.g. cutaneous SVV, testicular arteritis, central nervous system vasculitis). Vasculitis distribution may be unifocal or multifocal (diffuse) within an organ. Some patients originally diagnosed with SOV will develop additional disease manifestations that warrant re-defining the case as one of the systemic vasculitides (e.g. cutaneous arteritis later becoming systemic polyarteritis nodosa, etc.).

Index terms

  • Single organ vasculitis

Exclusions from above levels

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

Sibling codes

  • 4A44.0 – Rhizomelic pseudopolyarthritis
  • 4A44.1 – Aortic arch syndrome
  • 4A44.2 – Giant cell arteritis
  • 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 entity

Single organ vasculitis – http://id.who.int/icd/entity/1314272220

Part of: 4A44: Vasculitis › 04: Diseases of the immune system