ICD-11 code : 4A44.7;
Preferred Label : Primary angiitis of the central nervous system;
ICD-11 definition : 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.;
ICD-11 synonym : PACNS - [primary angiitis of the central nervous system]; Primary vasculitis of the central nervous system; Isolated angiitis of the central nervous system;
ICD-11 acronym : PACNS;
Origin ID : 2130738127;
Automatic exact mappings (from CISMeF team)
- ICD-10 Mapping
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.
