Juvenile Dermatomyositis

Zusammenfassung

Die juvenile Dermatomyositis (JDM) ist die häufigste chronische entzündliche Myopathie des Kindesalters. In dieser Übersicht soll der aktuelle Kenntnisstand hinsichtlich der Diagnostik, Behandlung und Überwachung der JDM dargestellt werden. So liegen häufig myositis-spezifische Antikörper vor, die mit klinischen Phänotypen und dem Verlauf der Erkrankung korrelieren. Typ I Interferone spielen eine wichtige Rolle in der Pathogenese der Erkrankung. Möglicherweise kann diese Beobachtung in der Zukunft zu gezielten Therapien führen. Da langfristig schwerwiegende Komplikationen, wie z. B. Kalzinosen oder Lipodystrophie, drohen, besonders bei auf Dauer unzureichend kontrollierter Erkrankung, ist eine möglichst rasche und effektive Behandlung anzustreben. Zu diesem Zweck sollte eine intensive Remissionsinduktionstherapie, gefolgt von einer zielgerichteten Therapie angestrebt werden. Verschiedene validierte Messinstrumente stehen zur Verfügung, um den Verlauf der Erkrankung zu beurteilen. Die Pro-KIND-Initiative der Gesellschaft für Kinder- und Jugendrheumatologie hat Praxis- und Konsens-basiert in Deutschland sowohl eine diagnostische als auch eine Treat-to-Target-Behandlungsstrategie entwickelt. Im Rahmen nationaler und internationaler Kollaborationen soll sich die Behandlung der JDM in der Zukunft weiter verbessern.

Abstract

Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy of childhood. In this review, the current state of knowledge concerning diagnostic testing, treatment and monitoring of JDM are illustrated. Myositis-specific antibodies are frequently observed, which may correlate with the disease phenotype and course. Furthermore, type 1 interferons play an important pathophysiological role in JDM. This may potentially lead to targeted therapies in the future. In the context of uncontrolled disease activity, serious complications may arise in the long term, such as calcinosis or lipodystrophy. Rapid implementation of efficacious treatment is therefore desirable. For this purpose, an intensive remission induction regimen is usually implemented, followed by a treat-to-target strategy. Multiple validated monitoring tools are available in order to gauge treatment response. The Pro-KIND project of the German Society for Pediatric and Adolescent Rheumatology has established practice- and consensus-based diagnostic and treat-to-target strategies. In the context of national and international collaboration, the management of JDM should be improved in the future.

Schlüsselwörter

Juvenile Dermatomyositis - Entzündliche Myopathie - Myositisspezifische Antikörper

Key words

juvenile dermatomyositis - inflammatory myopathy - myositis-specific antibodies

• Literatur

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