Primary CNS Vasculitis - A Focussed Review on Treatment.

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.
Competing Interests: 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.
(© 2024 Kesav et al.)