Primary Angiitis of the Central Nervous System

Rheum Dis Clin North Am. 2017 Nov;43(4):503-518. doi: 10.1016/j.rdc.2017.06.001.


Primary angiitis of the central nervous system (PACNS) is rare but often included in the differential diagnosis for unusual or unexplained neurologic symptoms owing to its array of clinical manifestations. PACNS is not a single entity, but rather an umbrella term for distinct subtypes of disease affecting different parts of the CNS, different sized vessels, and with different histologic and pathologic findings. Although brain biopsy is considered the gold standard test, it is neither highly sensitive nor specific. One must always pursue a workup for mimics of PACNS. Treatment consists of prednisone monotherapy or combination therapy with prednisone and cyclophosphamide.

Keywords: Amyloid beta-related angiitis; Brain biopsy; Granulomatous angiitis of the CNS; Neuroimaging; Primary angiitis of the central nervous system; Reversible cerebral vasoconstriction syndromes.

Publication types

  • Review

MeSH terms

  • Anti-Inflammatory Agents / therapeutic use
  • Antirheumatic Agents / therapeutic use
  • Humans
  • Magnetic Resonance Imaging
  • Vasculitis, Central Nervous System / complications
  • Vasculitis, Central Nervous System / diagnosis*
  • Vasculitis, Central Nervous System / therapy*


  • Anti-Inflammatory Agents
  • Antirheumatic Agents

Supplementary concepts

  • Primary angiitis of the central nervous system