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Zeitschrift für Rheumatologie

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29.04.2022 | Juvenile Dermatomyositis | Übersichten

Dermatomyositis und juvenile Dermatomyositis

verfasst von: Dr. med. Frank Dressler, Prof. Dr. med. Britta Maurer

Erschienen in: Zeitschrift für Rheumatologie | Ausgabe 3/2023

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Zusammenfassung

Die Dermatomyositis (DM) ist eine entzündliche Multisystemerkrankung unklarer Ätiologie, die bereits im Kindesalter beginnen, aber auch erst im hohen Erwachsenenalter auftreten kann. Myalgien und Muskelschwäche können erst im Verlauf der Erkrankung auftreten oder bei manchen Formen sogar ganz fehlen. Klassische Zeichen an der Haut sind heliotroper Ausschlag, Wangenerythem, Gottron-Zeichen sowie Nagelbettgefäßveränderungen. Bei der Diagnosestellung spielen v. a. auch zunehmend die Bestimmung Myositis-spezifischer Autoantikörper eine Rolle. Die Muskelenzyme können erhöht sein, sind es jedoch bei ca. einem Drittel der Patienten nicht. In Absenz typischer klinischer und serologischer Befunde können zur Diagnosestellung weitere Untersuchungsmethoden, wie z. B. Kapillarmikroskopie, Magnetresonanztomographie, Elektromyographie, Haut- oder Muskelbiopsie, in Betracht gezogen werden. Je nach klinisch-serologischem Subtyp empfiehlt sich auch ein gastrointestinales oder kardiopulmonales Organscreening. Bei erwachsenen Patienten sollte zusätzlich auch eine altersadaptierte Tumorsuche durchgeführt werden. Neben Kortikosteroiden zur Induktionstherapie und konventionellen Basistherapeutika und Immunglobulinen spielen zunehmend auch Biologika und Small-molecule-Inhibitoren eine Rolle. Die Prognose der DM/JDM (juvenile Dermatomyositis) hat sich verbessert. Während sich die meisten Patienten zumindest partiell erholen, versterben einzelne, und eine Minderheit entwickelt eine persistierende Muskelatrophie oder eine ausgeprägte Kalzinose.

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Titel: Dermatomyositis und juvenile Dermatomyositis
verfasst von: Dr. med. Frank Dressler
Prof. Dr. med. Britta Maurer
Publikationsdatum: 29.04.2022
Verlag: Springer Medizin
Schlagwörter: Juvenile Dermatomyositis
Diagnostik in der Rheumatologie
Dermatomyositis
Erschienen in: Zeitschrift für Rheumatologie / Ausgabe 3/2023
Print ISSN: 0340-1855
Elektronische ISSN: 1435-1250
DOI: https://doi.org/10.1007/s00393-022-01205-5

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