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Therapeutisch kommen in erster Linie Immunsuppressiva zum Einsatz, insbesondere Cyclophosphamid (CYC) und Mycophenolat-Mofetil (MMF). Neuere Daten zeigen zudem eine Wirksamkeit der Biologika Rituximab und Tocilizumab. Die therapeutischen Optionen wurden zuletzt durch die Zulassung des Antifibrotikums Nintedanib erweitert, dessen Stellenwert insbesondere bei den trotz Immunsuppression progredient fibrosierenden Verl\u00e4ufen der ILD liegt. Die in dieser \u00dcbersicht zusammengestellten Daten aus kontrollierten Studien zur Wirksamkeit und Sicherheit von CYC und MMF sprechen f\u00fcr einen bevorzugten Einsatz von MMF. Dem entgegen steht die noch immer fehlende Zulassung von MMF f\u00fcr diese Indikation. 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