Updates on efficacy and safety janus kinase inhibitors in juvenile dermatomyositis.

INTRODUCTION: Juvenile dermatomyositis (JDM) is a rare autoimmune disease most commonly with proximal weakness due to inflammation and characteristic skin rashes. Most patients have a chronic or polycyclic disease course on standard therapy so better treatments are needed.An interferon signature is well-established in key tissues of JDM. Janus kinase inhibitors (jakinibs), which can decrease IFN signaling, are therefore appealing as a targeted therapy.

AREAS COVERED: Herein is a review of the growing literature on JDM patients on jakinibs, including specifics of their jakinib exposure, summary of efficacy, disease features and characteristics of patients treated, and safety parameters.

EXPERT OPINION: The vast majority of refractory JDM patients respond to jakinib therapy, though they have varied features, doses, and previous/concurrent medications, though data is largely retrospective. Jakinibs are an exciting and promising treatment in JDM. Evaluation with larger prospective controlled studies is needed to answer remaining questions about jakinibs in JDM regarding dosing, which JDM patients to treat with jakinibs, potential biomarkers to use, and how best to monitor safety risks in JDM.