What are the recommendations for treating primary CNS angiitis with high-dose steroids?
In cases with confirmed primary CNS angiitis, administration of high-dose steroids is supported by observational data. Additionally, 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 it is recommended that an interdisciplinary follow-up is done for patients with primary CNS angiitis and that the choice of steroid-sparing agent be made considering the profile of each agent and associated comorbidities. 