Dermatomyositis, Antisynthetase-Syndrom und immunvermittelte nekrotisierende Myopathie

Zusammenfassung

Die inflammatorischen Myopathien werden heutzutage anhand von Klinik, Biopsie und Antikörperstatus genauer gegliedert. Zumeist sind sowohl die Dermatomyositis, das Antisynthetase-Syndrom (ASyS) und die Immunvermittelte nekrotisierende Myopathie (IMNM) durch proximale Muskelschwäche gekennzeichnet, wobei jedoch einige Entitäten auch amyopathisch verlaufen können. Je nach vorliegender Antikörperart kann die Dermatomyositis jeweils unterschiedliche Manifestationen aufweisen, wobei die interstitielle Lungenerkrankung (ILD) als häufigste extramuskuläre Beteiligung zu erwähnen ist. Ein besonderes Augenmerk sollte auf das Tumorscreening bei Vorliegen entsprechender Risikofaktoren, wie beispielsweise bei anti-TIF1γ oder anti-NXP2-positivem Antikörperstatus bei Erwachsenen, gelegt werden. Die IMNMs sind bekanntermaßen mit einer Statinexposition assoziiert, allerdings sollte bedacht werden, dass eine Statinexposition nicht zwingend nötig ist, um eine IMNM zu entwickeln. Das ASyS präsentiert sich regulär mit einer Triade aus Myositis, Arthritis und ILD, welche jedoch nicht immer vorliegen muss. Therapeutisch unterscheiden sich die drei Gruppen initial nicht. Eine überbrückende Prednisolontherapie (je nach Schweregrad oral oder intravenös) wird initiiert und je nach Grunderkrankung und Organbeteiligung parallel eine steroidsparende Erhaltungstherapie etabliert. Eine ergänzende Physiotherapie sowie bei Bedarf auch Ergo- oder Logopädie sind wichtige Bausteine der Therapie und sollten frühzeitig begonnen werden.

Abstract

Today, idiopathic inflammatory myopathies are categorised more precisely based on clinical findings, histopathological aspects, and autoantibody status, e. g., into dermatomyositis, antisynthetase syndrome, and immune-mediated necrotising myopathy (IMNM). These mostly present with proximal muscle weakness, although the disease can also occur in an amyopathic form in some cases. Dermatomyositis patients may present with varying manifestations, depending on the antibody status, with interstitial lung disease (ILD) being the most common extramuscular presentation. Especially in the presence of concomitant risk factors such as anti-TIF1γ or anti-NXP2-positive antibodies, a tumour screening should be performed in adult patients. IMNMs are known as being associated with exposure to statins, but it should be borne in mind that statin exposure is not a precondition for the development of an IMNM. Antisynthetase syndrome usually presents with a triad of myositis, arthritis, and ILD. Here, too, the classical triad is not necessarily present. As regards therapeutic approaches, the three entities do not initially differ. An initial steroid therapy is started and complemented by steroid-sparing maintenance therapy, which depends on the severity and organ involvement of the disease. Physiotherapy and, if needed, occupational therapy and logopaedics are integral parts of the treatment and should be initiated at an early point in time.

Schlüsselwörter

Dermatomyositis - Antisynthetase-Syndrom - Immunvermittelte nekrotisierende Myopathie - Autoantikörper - Idiopathisch inflammatorische Myopathie

Keywords

dermatomyositis - antisynthetase syndrome - autoantibody - idiopathic inflammatory myopathy - immune-mediated necrotising myopathy

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