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29.12.2023 | Juvenile Dermatomyositis | Leitthema

Fortschritte in der Diagnostik und Therapie der juvenilen Dermatomyositis

verfasst von: Ingrid Georgi, Mathias Georgi, Prof. Dr. med. Johannes-Peter Haas

Erschienen in: Zeitschrift für Rheumatologie | Ausgabe 1/2024

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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.

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Titel: Fortschritte in der Diagnostik und Therapie der juvenilen Dermatomyositis
verfasst von: Ingrid Georgi
Mathias Georgi
Prof. Dr. med. Johannes-Peter Haas
Publikationsdatum: 29.12.2023
Verlag: Springer Medizin
Schlagwörter: Juvenile Dermatomyositis
Myopathien
Dermatomyositis
Erschienen in: Zeitschrift für Rheumatologie / Ausgabe 1/2024
Print ISSN: 0340-1855
Elektronische ISSN: 1435-1250
DOI: https://doi.org/10.1007/s00393-023-01454-y

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