Abstract
Inflammatory brain diseases, including childhood primary CNS vasculitis, are increasingly recognized disease entities. Mortality and morbidity have improved markedly due to rapid recognition and initiation of treatment. Treatment regimens are mostly derived from the adult literature. In this review, we summarize the most important adult and pediatric literature on therapy of primary CNS vasculitis. Evidence is all based on prospective or retrospective studies, as no trials have been performed in CNS vasculitis. Future new biomarker discoveries and increased knowledge on the pathophysiology are key for developing targeted treatment regimens.
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