Juvenile Dermatomyositis und andere idiopathische inflammatorische Myopathien des Kindesalters

ZUSAMMENFASSUNG

Die juvenile Dermatomyositis (JDM) ist die häufigste idiopathische inflammatorische Myopathie (IIM) des Kindes- und Jugendalters. Charakteristische Hautveränderungen bei der JDM sind ein symmetrisches Wangenerythem, heliotroper Ausschlag, Gottron-Zeichen sowie Nagelbettgefäßveränderungen. Andere IIM-Formen des Kindesalters sind das Anti-Synthetase-Syndrom (ASyS), immunvermittelte nekrotisierende Myopathie (IMNM) oder Overlap-Syndrome. Bei der Diagnosestellung und Kategorisierung hat die Bestimmung Myositis-spezifischer Antikörper zunehmend Bedeutung. Zur Diagnosestellung werden weitere Parameter und Untersuchungsmethoden wie Muskelenzyme, Immunaktivierungsmarker, Magnetresonanztomografie, Kapillarmikroskopie, Elektromyografie und/oder Haut- oder Muskelbiopsie herangezogen. Je nach klinisch-serologischem Subtyp kann eine weitere Organdiagnostik notwendig sein. Therapeutisch werden Glukokortikoide hochdosiert zur Induktionstherapie und mehrjährig Basistherapeutika wie Methotrexat, Ciclosporin oder andere Immunsuppressiva sowie Immunglobuline eingesetzt. Januskinase-Inhibitoren sind vielversprechend, da sie effektiv Interferon-Signalwege hemmen können. Physiotherapie und Widerstandstraining helfen, Kontrakturen und dem Verlust von Muskelmasse entgegenzuwirken. Die Prognose der IIM hat sich deutlich verbessert, dennoch sind Spätfolgen, wie z. B. eine persistierende Muskelatrophie, Kontrakturen oder Kalzinose relativ häufig.

Schlüsselwörter

Juvenile Dermatomyositis - idiopathische inflammatorische Myopathie - Myositis-spezifische Autoantikörper - Kalzinose

Publikationsverlauf

Artikel online veröffentlicht:
28. Februar 2024

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