Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2024 Oct 24:20:453-465.
doi: 10.2147/VHRM.S488202. eCollection 2024.

Primary CNS Vasculitis - A Focussed Review on Treatment

Praveen Kesav  1 , Divya Manesh Raj  2 , Rula A Hajj-Ali  3 , Syed I Hussain  4 , Seby John  4
Affiliations
Review

Primary CNS Vasculitis - A Focussed Review on Treatment

Praveen Kesav et al. Vasc Health Risk Manag. .

Abstract

Primary central nervous system vasculitis (PCNSV) is a rare and complex disease that poses formidable diagnostic and therapeutic challenges. Since its initial recognition as a distinct clinical entity in the 1950s, there has been considerable advancement in our understanding of PCNSV histopathology, specific clinical subsets, and their response to treatment. However, PCNSV is one of the rarest vasculitides, and still remains a challenging diagnosis with many unanswered questions regarding optimal management. In this review, we intend to provide a detailed outline of approaches that are currently being employed for the treatment of PCNSV. We exhaustively review available cohort series of PCNSV and critically appraise the data for study definitions, treatment approaches, and predictors of treatment outcomes. Finally, we also propose a treatment approach for PCNSV.

Keywords: PACNS; PCNSV; induction; maintenance; outcomes; primary CNS vasculitis; primary angiitis of CNS; relapse; remission; treatment.

PubMed Disclaimer

Conflict of interest statement

Dr Rula Hajj-Ali reports personal fees from GSK, personal fees from Amgen, personal fees from UptoDate, outside the submitted work. The authors report no other conflicts of interest in this work.

References

    1. Newman W, Wolf A. Non-infectious granulomatous angiitis involving the central nervous system. Trans Am Neurol Assoc. 1952;56:114–117. - PubMed
    1. Cravioto H, Feigin I. Non-infectious granulomatous angiitis with a predilection for the nervous system. Neurology. 1959;9(9):599–609. doi:10.1212/WNL.9.9.599 - DOI - PubMed
    1. Salvarani C, Brown RD, Calamia KT, et al. Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol. 2007;62(5):442–451. doi:10.1002/ana.21226 - DOI - PubMed
    1. Byram K, Hajj-Ali RA, Calabrese L. CNS Vasculitis: an approach to differential diagnosis and management. Curr Rheumatol Rep. 2018;20(7):37. PMID: 29846828. doi:10.1007/s11926-018-0747-z - DOI - PubMed
    1. John S, Hajj-Ali RA. CNS vasculitis. Semin Neurol. 2014;34(4):405–412. PMID: 25369436. doi:10.1055/s-0034-1390389 - DOI - PubMed

Substances

LinkOut - more resources