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Fortschritte in der Diagnostik und Therapie der juvenilen Dermatomyositis

Advances in the diagnostics and treatment of juvenile dermatomyositis

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Zusammenfassung

Die juvenile Dermatomyositis (JDM) ist im Kindes- und Jugendalter die mit Abstand häufigste inflammatorische Myopathie. Klinisch gekennzeichnet durch entzündliche Haut- und Muskelveränderungen, kann sie als Multisystemerkrankung auch das Skelettsystem, den Gastrointestinaltrakt, Herz und Lunge befallen. Bei der Pathogenese spielen intrinsische (multigenetisches Risiko) und extrinsische Faktoren (Trigger) eine Rolle und führen zu Endothelschäden, einer Faszienbeteiligung, einer Aktivierung des Interferonsystems und zu Autoimmunreaktionen inklusive Bildung Myositis-spezifischer-Autoantikörper (MSA). Im Unterschied zur Dermatomyositis (DM) bei Erwachsenen besteht bei Kindern und Jugendlichen keine Assoziation zu malignen Erkrankungen. Die variable Ausprägung, die Seltenheit der Erkrankung und das Risiko von Langzeitschädigungen und Komplikationen erfordern kinderrheumatologische Erfahrung bei Diagnostik und Therapie. Neue medikamentöse Möglichkeiten haben die Prognose und das Outcome erheblich verbessert, aber gerade initial ist eine multidisziplinäre Versorgung (Ärzte, Physiotherapeuten, Psychologen, Sozialarbeiter) erforderlich. Besonders wichtig ist eine fachgerechte Behandlung der funktionellen Folgen, die in diesem Beitrag besonders fokussiert wird.

Abstract

Juvenile dermatomyositis (JDM) is by far the most frequent inflammatory myopathy in childhood and adolescence. It is clinically characterized by inflammatory changes of the skin and muscles but as a multisystemic disease can also affect the skeletal system, the gastrointestinal tract, lungs and heart. Intrinsic (multigenetic risk) and extrinsic factors (triggers) are involved in the pathogenesis resulting in endothelial damage, involvement of fascies, activation of the interferon system and autoimmune reactions including formation of myositis-specific autoantibodies (MSA). In contrast to dermatomyositis in adults, in children and adolescents there are no associations with malignant diseases. The variable expression, the rarity of the disease and the risk of long-term damage and complications necessitate pediatric rheumatological experience in the diagnostics and treatment. Recently, new approaches in drug treatment have substantially improved the outcome and prognosis but a multidisciplinary treatment (including physicians, physiotherapists, psychologists, social workers) is mandatory, especially in the first phases of the disease. Particularly important is a professionally correct treatment of the functional sequelae, which are a particular focus of this article.

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  1. Deutsches Zentrum für Kinder- und Jugendrheumatologie, Kinderklinik Garmisch-Partenkirchen, Gehfeldstr. 24, 82467, Garmisch-Partenkirchen, Deutschland

    Ingrid Georgi, Mathias Georgi &  Johannes-Peter Haas

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Georgi, I., Georgi, M. & Haas, JP. Fortschritte in der Diagnostik und Therapie der juvenilen Dermatomyositis. Z Rheumatol 83, 41–51 (2024). https://doi.org/10.1007/s00393-023-01454-y

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