4A44.7

ICD-11 MMS 2026-01

Primary angiitis of the central nervous system

Description

In primary angiitis of the central nervous system, vasculitis is limited to the central nervous system. Primary angiitis of the central nervous system is a very rare disease, and its manifestation can be mimicked by many other diseases. Patients with primary angiitis commonly show headache, waxing and waning altered mental status, and transient ischemic attack-like events. Diagnosis is often based on angiography, although brain biopsy remains the only definitive diagnostic test.

Index terms

  • Primary angiitis of the central nervous system
  • Isolated angiitis of the central nervous system
  • PACNS - [primary angiitis of the central nervous system]
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  • Primary vasculitis of the central nervous system

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
  • 4A44.3 – Single organ vasculitis
  • 4A44.4 – Polyarteritis nodosa
  • 4A44.5 – Mucocutaneous lymph node syndrome
  • 4A44.6 – Sneddon syndrome
  • 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

Primary angiitis of the central nervous system – http://id.who.int/icd/entity/2130738127

Allowed postcoordination axes

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