Update Therapie der ANCA-assoziierten Vaskulitiden

Zusammenfassung

Dieser Artikel gibt eine Übersicht über die aktuellen deutschen und europäischen Therapieempfehlungen zu den ANCA-assoziierten Vaskulitiden (AAV) sowie ein Update zu seither publizierten wichtigen Therapiestudien. Nach den deutschen und europäischen Empfehlungen erfolgt die Remissionsinduktion bei GPA/MPA mit organbedrohender Erkrankung mit Glucocorticoiden und Cycloposphamid oder Rituximab, bei nicht organbedrohender Erkrankung mit Glucocorticoiden und Methotrexat (MTX) oder Mycophenolat-Mofetil (MMF). Die kürzlich publizierte MYCYC-Studie unterstützt die Empfehlungen; sie zeigte eine Nicht-Unterlegenheit gegenüber i. v. Cyclosphamid bei GPA/MPA-Patienten mit nicht-organbedrohender und organbedrohender Erkrankung auf. Die remissionserhaltende Therapie soll mit niedrig-dosierten Glucocorticoiden sowie Azathioprin, MTX und Rituximab durchgeführt werden. Rituximab wurde Ende 2018 auf der Basis der MAINRITSAN-Studie als remissionserhaltende Therapie zugelassen und ist damit nun die formal einzige zur Remissionserhaltung zugelassene Therapie. Mehrere Studien zur untersuchten zudem die kurze Anwendung von Glucocorticoiden (z. B. durch additive Therapie mit Avacopan) in der Remissionsinduktion und das komplette Ausschleichen von Glucocorticoiden in der Remissionserhaltung. Die ersten Ergebnisse hierzu sind vielversprechend; Langzeitdaten stehen allerdings aus. Für die sehr viel seltenere EGPA liegen insgesamt deutlich weniger Studiendaten vor. Die Therapie wird jedoch im Wesentlichen nach den gleichen Prinzipien wie bei den anderen AAV durchgeführt. Neuerdings steht, in Deutschland für diese Indikation aber noch nicht zugelassen, eine Biologikatherapie mit dem Anti-IL-5-Antikörper Mepolizumab zur Verfügung.

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.

Schlüsselwörter

ANCA-Assoziierte Vaskulitis - Granulomatose mit Polyangiitis - Mirkoskopische Polyangiitis - Eosinophile Granulomatose mit Polyangiitis - Rituximab

Key words

ANCA-associated vasculitis - Rituximab - granulomatosis with polyangiitis - microscopic polyangiitis - eosinophilic granulomatosis with polyangiitis

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