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Praveen Kesav,1 Divya Manesh Raj,2 Rula A Hajj-Ali,3 Syed I Hussain,4 Seby John4 1Department of Neurology, Neurological Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates; 2Research Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates; 3Department of Rheumatic and Immunologic Diseases, Center for Vasculitis Care and Research, Cleveland Clinic Lerner College of Medicine, Cleveland, OH, USA; 4Department of Neuro-Interventional Surgery, Neurological Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab EmiratesCorrespondence: Seby John, Email johns5@clevelandclinicabudhabi.aeAbstract: 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: primary CNS vasculitis, primary angiitis of CNS, PCNSV, PACNS, treatment, induction, maintenance, remission, relapse, outcomes |
