Juvenile dermatomyositis

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.