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Pediatric Rheumatology
Published in: Pediatric Rheumatology 1/2023

Open Access 01-12-2023 | Juvenile Dermatomyositis | Case Report

Rapid and sustained response to JAK inhibition in a child with severe MDA5 + juvenile dermatomyositis

Authors: Timmy Strauss, Claudia Günther, Anja Schnabel, Christine Wolf, Gabriele Hahn, Min Ae Lee-Kirsch, Normi Brück

Published in: Pediatric Rheumatology | Issue 1/2023

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Abstract

Background

Juvenile dermatomyositis (jDM) is the most common idiopathic inflammatory myopathy of childhood. Amyopathic or hypomyopathic courses have been described.

Case presentation

We present the case of a 4-year-old patient with MDA5 antibody positive jDM and interstitial lung disease. In our patient, typical symptoms of jDM with classical skin lesions, arthritis, proximal muscle weakness, and ulcerative calcifications were observed. Due to the severity of the disease and the pulmonary changes, therapy with the Janus kinase (JAK) inhibitor ruxolitinib was added to the therapy with corticosteroids, intravenous immunoglobulins (IVIG) and hydroxychloroquine leading to a fast and sustained remission.

Conclusion

While there is growing evidence that JAK inhibition is a promising therapeutic option in jDM our case report shows that this approach may also be effective in MDA5-positive jDM with high risk features.
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Metadata
Title
Rapid and sustained response to JAK inhibition in a child with severe MDA5 + juvenile dermatomyositis
Authors
Timmy Strauss
Claudia Günther
Anja Schnabel
Christine Wolf
Gabriele Hahn
Min Ae Lee-Kirsch
Normi Brück
Publication date
01-12-2023
Publisher
BioMed Central
Published in
Pediatric Rheumatology / Issue 1/2023
Electronic ISSN: 1546-0096
DOI
https://doi.org/10.1186/s12969-023-00894-9

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