Update on the Treatment of ANCA-associated Vasculitis

Abstract

This article provides an overview of current German and European treatment guidelines for ANCA-associated vasculitis (AAV) and an update on major published studies. According to German and European guidelines, patients with organ-threatening GPA/MPA should be treated with glucocorticoids and either cyclophosphamide or rituximab. Patients with non-organ-threatening disease should receive glucocorticoids and methotrexate (MTX) or mycophenolate (MMF). The MYCYC study, which has recently been published, supports the use of MMF. This study demonstrated non-inferiority compared with cyclophosphamide in patients with non-organ-threatening and organ-threatening disease. Remission maintenance should be performed with low-dose glucocorticoids and either azathioprine, MTX or rituximab. Rituximab was approved for remission maintenance at the end of 2018. It is the only drug approved for remission maintenance in GPA/MPA. Several studies have investigated the short-term use of glucocorticoids during remission induction in GPA/MPA (i. e. by additive treatment with Avacopan) and the complete tapering of glucocorticoids during remission. The first results are promising, while long-term data are still pending. There is much less evidence from clinical trials concerning the treatment of EGPA. In principle, however, the treatment strategies are comparable to those governing the treatment of other AAVs. A biological treatment option with the IL-5 antibody mepolizumab has recently become available, but this treatment has not yet been approved in Germany.