Therapieempfehlungen bei IgG4-assoziierter Erkrankung

Zusammenfassung

Die IgG4-assoziierte Erkrankung (englisch: IgG4-related disease, IgG4-RD) stellt eine seltene, fibro-inflammatorische Erkrankung dar, deren Therapie aufgrund der begrenzten Studienlage und fehlender zugelassener Medikamente zunächst auf Erfahrungswerten basierte und über die Zeit eine immer bessere Evidenzgrundlage erhält. Nach der klinischen Einschätzung, ob eine systemische Therapie notwendig ist, wird bei symptomatischen Patienten in der Regel eine Remissionsinduktion mit oraler Glukokortikoidtherapie durchgeführt. Die Remissionserhaltung erfolgt durch schrittweise Dosisreduktion, wobei zunächst eine Erhaltungsdosis von 2,5 bis 7,5 mg/Tag angestrebt wird. Bei häufigen Rezidiven oder ungünstigen Prognosefaktoren ist der Einsatz von Steroid-sparenden Medikamenten wie Azathioprin, Methotrexat oder Rituximab erforderlich, um die Kortisondosis zu minimieren und Folgeschäden zu vermeiden. Aktuelle Studien zeigen, dass eine Kombinationstherapie aus Prednisolon und Immunmodulatoren vorteilhaft sein kann. Neuere Entwicklungen in der Therapie, wie der Einsatz von Inebilizumab, einem monoklonalen Antikörper, zeigen vielversprechende Ergebnisse in der Reduktion der Rezidivrate.

Abstract

IgG4-related disease (IgG4-RD) is a rare fibro-inflammatory rheumatic disease. Its treatment is initially based on clinical experience - due to the limited studies and lack of approved medications. Nevertheless, the evidence base is growing over time. After clinical assessment of the necessity for systemic therapy, symptomatic patients typically undergo remission induction with oral glucocorticoid therapy. Remission maintenance is achieved through gradual dose reduction, which initially aims for a maintenance dose of 2.5 to 7.5 mg/day. In cases of frequent relapses or unfavourable prognostic factors, the use of steroid-sparing agents such as azathioprine, methotrexate, or rituximab is necessary, in order to minimise corticosteroid dosage and prevent side effects. Current studies indicate that combination therapy with prednisone and immunomodulators may be beneficial. Recent developments in treatment, such as the use of Inebilizumab, a monoclonal antibody, show promising results in reducing relapse rates.

Schlüsselwörter

IgG4-assoziierte Erkrankung - Therapieempfehlungen - Übersichtsartikel

Keywords

IgG4-related disease - therapy guidelines - therapy recommendations - review - treatment

Publication History

Received: 17 February 2025

Accepted: 28 May 2025

Article published online:
14 July 2025

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