CNS Vasculitis: an Approach to Differential Diagnosis and Management

Curr Rheumatol Rep. 2018 May 30;20(7):37. doi: 10.1007/s11926-018-0747-z.


Purpose of review: The goal of this review is to provide an up-to-date approach to diagnosis and management of patients with central nervous system (CNS) vasculitis.

Recent findings: Challenges in diagnosis of CNS vasculitis still exist due to the broad differential diagnosis and generally nonspecific initial clinical manifestations. Differentiation between primary angiitis of the CNS (PACNS) and secondary causes is important in guiding management. Recent longitudinal cohort studies have improved our understanding of PACNS. Advances in neuroimaging and molecular testing have enhanced diagnostic decision-making. Therapy remains largely empiric, guided by observational data. Despite the limited use of targeted therapies, glucocorticoids and cyclophosphamide remain the mainstays of therapy in PACNS. Securing a diagnosis through a careful, team-based approach with emphasis on ruling out possible mimics is paramount in the management of patients with CNS vasculitis.

Keywords: CNS vasculitis; Primary CNS vasculitis; Primary angiitis of the central nervous system; Primary central nervous system vasculitis.

Publication types

  • Review

MeSH terms

  • Cyclophosphamide / therapeutic use
  • Diagnosis, Differential
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Vasculitis, Central Nervous System / diagnosis*
  • Vasculitis, Central Nervous System / drug therapy


  • Glucocorticoids
  • Immunosuppressive Agents
  • Cyclophosphamide

Supplementary concepts

  • Primary angiitis of the central nervous system