What are the recommended treatments for primary CNS angiitis?
In cases with confirmed primary CNS angiitis, administration of high-dose steroids is supported by observational data. Slow tapering of steroids followed by the addition of a steroid-sparing agent is preferred over long-term monotherapy with steroids due to a higher rate of relapse and poorer outcomes with steroid monotherapy. Some commonly used maintenance steroid-sparing agents include cyclophosphamide and azathioprine. There are no robust data related to efficacy between these agents, so an interdisciplinary follow-up for patients with primary CNS angiitis is recommended, and the choice of steroid-sparing agent should be made considering the profile of each agent and associated comorbidities. 