CNS Vasculitis: an Approach to Differential Diagnosis and Management

Current Rheumatology Reports
Kevin ByramLeonard H Calabrese

Abstract

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. 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.

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Citations

Apr 19, 2019·Internal Medicine·Keigo AshitaniHiroyuki Imaeda
Feb 23, 2020·Stroke; a Journal of Cerebral Circulation·Neal S ParikhCostantino Iadecola
Oct 28, 2019·Practical Neurology·Claire M Rice, Neil J Scolding
Oct 28, 2019·Current Opinion in Rheumatology·Rula A Hajj-Ali, Leonard H Calabrese
Jan 6, 2019·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·AnneMarie M Denny, Sumit K Das
May 27, 2021·Scientific Reports·L A DobryninaM V Krotenkova
Jul 2, 2021·Current Rheumatology Reports·Joana Martins-MartinhoCristina Ponte
Dec 7, 2021·Acta Neurologica Scandinavica·Verónica CabreiraElsa Azevedo

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