ICD-11

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.

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.