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Physikalische Medizin, Rehabilitationsmedizin, Kurortmedizin 2017; 27(01): 23-34
DOI: 10.1055/s-0042-123877
Übersicht
© Georg Thieme Verlag KG Stuttgart · New York

Entzündliche Muskelerkrankungen – Aktueller Stand zur Diagnostik und Therapie der Myositis

Inflammatory Muscle Disorders – State of the Art of Diagnosis and Treatment of Myositis
Maren Fitzner
1   Klinik für Neurologie, Universitätsmedizin Göttingen, Göttingen
,
Jens Schmidt
1   Klinik für Neurologie, Universitätsmedizin Göttingen, Göttingen
2   Institut für Neuroimmunologie, Institut für Multiple-Sklerose-Forschung und Hertie Stiftung, Universitätsmedizin Göttingen
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Publication History

Publication Date:
12 April 2017 (online)

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Zusammenfassung

Bei den idiopathischen inflammatorischen Myopathien unterscheidet man die Polymyositis (PM), Dermatomyositis (DM), nekrotisierende Myopathie (NM), Einschlusskörpermyositis (inclusion body myositis = IBM) und Myositis bei Overlap-Syndrom. Es handelt es sich um eine heterogene Gruppe erworbener Muskelerkrankungen, die sich durch eine progrediente Muskelschwäche und Muskelatrophien auszeichnet. Die Abgrenzung der einzelnen Subtypen hat insbesondere im Hinblick auf die Prognose und Wahl der Therapie eine wesentliche Bedeutung. Die Identifikation muskelspezifischer Antikörper hat in den letzten Jahren die Diagnostik wesentlich vorangebracht, jedoch bleibt die Muskelbiopsie weiterhin ein unverzichtbares Hauptelement in der differentialdiagnostischen Abklärung von Myositissyndromen. Die Therapieempfehlungen basieren in Anbetracht des Mangels hochqualitativer Studien weiterhin überwiegend auf empirischen Daten und Expertenmeinungen und es sind erstmals fachübergreifende Leitlinien mit Beteiligung von Dermatologen, Rheumatologen und Neurologen entwickelt worden. Während bei der PM, DM und NM immunsuppressive Therapien zum Einsatz kommen, scheint die IBM gegenüber diesen Ansätzen weitestgehend refraktär zu sein. Hierdurch wird deutlich, dass auch die Abgrenzung zu anderen, nicht-entzündlichen Muskelerkrankungen essenziell ist. Die vorliegende Übersicht stellt den aktuellen state-of-the-art zur Diagnostik und Therapie von Myositiden dar.

Abstract

Idiopathic inflammatory myopathies include polymyositis (PM), dermatomyositis (DM), necrotizing myopathy (NM), inclusion body myositis (IBM), and myositis in overlap syndromes. This is a heterogeneous group of acquired muscle diseases characterised by progressive muscle weakness and muscular atrophy. It is crucial to differentiate between the individual subtypes, especially with a view to prognosis and choice of treatment. Although the identification of muscle-specific antibodies has significantly advanced the diagnostic process in the past few years, muscle biopsy remains an indispensable element in the differential-diagnostic workup of myositis syndromes. Due to the lack of high-quality studies, most treatment recommendations continue to be based on empirical data and expert opinions, and the first interdisciplinary guidelines have been developed jointly by dermatologists, rheumatologists and neurologists. While patients with PM, DM and NM are eligible for immunosuppressive treatment, IBM seems to be largely refractory to this approach. This illustrates that it is also essential to distinguish between myositis and other, non-inflammatory muscle diseases. The following review article presents the current state-of-the-art approach to the diagnostic workup and treatment of myositis.

Schlüsselwörter

Myositis - inflammatorische Myopathien - Dermatomyositis - Polymyositis - Overlap-Syndrom - Nekrotisierende Myopathie - Einschlusskörpermyositis

Key words

myositis - inflammatory myopathies - dermatomyositis - polymyositis - overlap syndrome - necrotizing myopathy - inclusion body myositis
 

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