Vasculitis is a rare but severe disease. It is diagnostically challenging because patients present with nonspecific symptoms. The definitive diagnosis is therefore complicated, because many diagnostic procedures have to be performed. Currently, several systemic types are known that have a secondary affect on the nervous system; an isolated primary central nervous system (CNS) vasculitis also occurs. In this review, we highlight new developments in medical treatment and provide an overview of the various combination strategies regarding the most significant types of vasculitis.
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