Moderne Immuntherapien: Myositis

Zusammenfassung

Myositiden sind heterogene Autoimmunerkrankungen mit potenziell schwerem Verlauf und Organbeteiligung wie Lunge oder Herz. Basistherapien (z. B. Glukokortikoide, AZA, MTX) zeigen oft unzureichendes Ansprechen, weshalb eine frühe, antikörperspezifische Therapieeskalation empfohlen wird. IVIG, Rituximab, JAK-Inhibitoren und neuere Ansätze wie CAR-T-Zellen oder CD38-/BCMA-gerichtete Therapien bieten individuelle Optionen bei therapierefraktären Verläufen. Die Organbeteiligung, der klinischer Verlauf und die Pathophysiologie (z. B. IFN-I-Signatur) sind wichtige Kriterien eine Entscheidung zur Therapieeskalation. Eine interdisziplinäre Fallbesprechung und eine individuelle Risiko-Nutzen-Abwägung sind essenziell, um Komplikationen zu minimieren, irreversible Muskelschäden zu vermeiden und eine langfristige Krankheitskontrolle zu erreichen.

Abstract

Myositis syndromes are heterogeneous inflammatory muscle diseases that often require individualized treatment based on organ involvement and antibody profiles. Initial therapy typically includes corticosteroids and immunosuppressants like azathioprine, methotrexate, or mycophenolate mofetil. In refractory cases, early escalation to IVIG, Rituximab, or JAK inhibitors is recommended. Specific manifestations such as interstitial lung disease or cardiac involvement necessitate targeted diagnostics and therapies. Experimental approaches, including FcRn inhibitors, CAR-T cells, CD38 antibodies, and BCMA-targeting agents, offer new hope for therapy-resistant cases. Due to limited study data and potential risks, therapy decisions should be made in interdisciplinary settings with individualized risk-benefit assessments.

Schlüsselwörter

Myositis - Inflammatorische Myopathie - Entzündliche Muskelerkrankung

Keywords

Myositis - Inflammatory myopathy - Inflammatory muscle disorder

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Publikationsverlauf

Artikel online veröffentlicht:
01. Dezember 2025

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