Diagnostik und Therapie der Myositis

Zusammenfassung

In den letzten Jahren gelingt es durch den Nachweis differenzierender Antikörper, subtilere bildgebende und histopathologische Diagnostik immer besser, die idiopathischen inflammatorischen Myopathien (IIM) einzuordnen. Neben der Polymyositis (PM) und Dermatomyositis (DM) sowie Einschlusskörperchenmyositis (IBM) grenzt sich das Anti-Synthetase-Syndrom ab. Nach auslösenden Ursachen wie myotoxischen Substanzen sollte besonders bei der nekrotisierenden Myositis gesucht werden. Ein Malignom-Ausschluss muss bei deutlich erhöhtem Risiko immer, insbesondere bei Nachweis von anti-TIF-1 gamma- und anti-NXP-2-Antikörpern erfolgen, dabei kann die Myositis dem Tumorleiden vorausgehen, sodass auch ein follow-up diesbezüglich erforderlich ist. Therapiestandard bleiben weiter die Glukokortikoide und Immunglobuline. Rituximab (RTX) stellt einen Meilenstein in der Therapie der schweren IIM dar. Auch wenn die RIM-Studie ihren primären Endpunkt verfehlt hat, zeigt sie wie, die inzwischen vorliegenden zahlreichen Fallberichte und Fallserien mit großen Patientenzahlen, das klare Ansprechen der meisten Patienten. Dabei benötigen viele Patienten nur einen Kurs (RTX), eine Reihe von Patienten erhält Kurse in großen Abständen bei sehr guter Verträglichkeit. In der Zukunft wird es noch besser gelingen, Subgruppen zu definieren und Responder und Nonresponder im Voraus zu erkennen. Auch für die IBM zeichnen sich nach Nachweis des ersten Antikörpers und den ersten Daten zu Bimagrumab erfolgversprechende neue Therapieoptionen ab. Eine Herausforderung bleibt das Management von Infektionen, der Dysphagie, der schweren Organbeteiligung sowie der Multimorbidität bei immer älter werdenden Patienten.

Abstract

The identification of differentiating antibodies and the use of more subtle imaging procedures and diagnostic histopathological procedures have allowed for idiopathic inflammatory myopathies (IIM) to be classified more accurately in the past few years. In addition to polymyositis, dermatomyositis and inclusion body myositis (IBM), the antisynthetase syndrome has been identified. Causative factors like myotoxic substances should be looked for especially in cases of necrotising myositis. Malignant diseases should always be ruled out in myositis patients, especially when anti-TIF1 gamma or anti-NXP-2 antibodies have been identified. As myositis may be a forerunner of a tumour, follow-up investigations are essentiell in this setting. Glucocorticoids and immunoglobulins still constitute the standard treatment for this condition. Rituximab (RTX) is a milestone in the treatment of severe IIM. Although the RIM study missed its primary endpoint, it has shown obvious responses in the majority of patients, as has also been demonstrated in several case reports and case series with large numbers of patients. Many patients need just one course of treatment (RTX). A number of patients receive several courses at large intervals and tolerate the treatment well. Subgroups will be defined even more specifically in the future, and it will be possible to differentiate responders from non-responders in advance. The detection of the first antibody and preliminary data on bimagrumab lead to the emergence of promising new treatment options for IBM. The management of infections, dysphagia, severe organ involvement and multimorbidity in an aging patient population continues to be a challenge in this context.

Schlüsselwörter

idiopathische inflammatorische Myopathie - Dermatomyositis - Polymyositis - Einschlusskörperchenmyositis - Therapiestandards

Key words

dermatomyositis - polymyositis - inclusion body myositis - standard treatment - idiopathic inflammatory myopathy

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