Secondary Logo

Journal Logo

Institutional members access full text with Ovid®

Management of primary and secondary central nervous system vasculitis

Salvarani, Carloa; Pipitone, Nicolòa; Hunder, Gene G.b

Current Opinion in Rheumatology: January 2016 - Volume 28 - Issue 1 - p 21–28
doi: 10.1097/BOR.0000000000000229
VASCULITIS SYNDROMES: Edited by Hasan Yazici and Yusuf Yazici

Purpose of review This article summarizes the current evidence on the management of primary and secondary central nervous system (CNS) vasculitis.

Recent findings Two recent retrospective cohort studies have described the treatment and outcomes of patients with adult primary central nervous system vasculitis (PCNSV). Although the majority of the patients (>60%) responded to therapy with glucocorticoids alone or in conjunction with cyclophosphamide (CYC) and tended to improve during the follow-up period, an overall increased mortality was observed. The treatment response and the outcomes appeared primarily related to the size of the vessels involved in the inflammatory process. The involvement of small cortical/leptomeningeal vessels was associated with a more benign course, whereas the involvement of larger/proximal cerebral vessels was related to a less favorable prognosis and identified cases that should be treated more aggressively. Glucocorticoids combined with CYC are the mainstay of therapy for secondary CNS vasculitis. Observational studies have documented the efficacy of rituximab as induction therapy in patients with antineutrophil cytoplasm antibody-associated vasculitis (AAV) and CNS disease, while the role of antitumor necrosis factor (TNF) agents is more controversial. Case series have demonstrated the efficacy of anti-TNF agents in patients with neuro-Behçet's disease. Tocilizumab may also be effective in this condition.

Summary Recognition of findings at diagnosis that predict the course or outcomes of PCNSV may serve as guide for therapy. Biological agents may provide benefit to difficult-to-treat patients with CNS involvement secondary to AAV and Behçet's disease.

aUnità Operativa di Reumatologia, Azienda Ospedaliera-IRCCS di Reggio Emilia, Reggio Emilia, Italy

bDivision of Rheumatology, Mayo Clinic, Rochester, Minnesota, USA

Correspondence to Carlo Salvarani, MD, Unità Operativa di Reumatologia, Azienda Ospedaliera-IRCCS di Reggio Emilia, V.le Risorgimento N80, 42100 Reggio Emilia, Italy. Tel: +39 0522 296616; fax: +39 0522 295836; e-mail:

Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved.