Juvenile Dermatomyositis: A Review of the Literature

Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy of childhood, characterized by proximal muscle weakness, pathognomonic cutaneous findings, and multisystem involvement. Over the past decade, significant advances have been made in pathogenesis and treatment of JDM. This review provides a comprehensive update on the etiopathogenesis, clinical features, autoantibody profiles, and treatment of JDM. Type I interferon signaling, vasculopathy, and mitochondrial dysfunction have emerged as interconnected pathogenic mechanisms, and their identification has informed the development of targeted therapeutic strategies. In particular, elucidation of the type I interferon pathway has contributed to disease diagnosis, monitoring of disease activity through interferon-related biomarkers, and the development of novel targeted therapies. Myositis-specific autoantibodies (MSA), detected in approximately 70% of patients, define clinically distinct subgroups; for example, anti-NXP2 is associated with calcinosis, and anti-MDA5 positivity carries a risk of interstitial lung disease. Importantly, MSA subtype influences not only clinical phenotype but also treatment response, underscoring the value of systematic autoantibody testing in guiding therapeutic decisions. A treat-to-target approach and close monitoring of disease activity are recommended. In refractory disease, conventional immunosuppressants and biologic agents are escalated sequentially, with Janus kinase (JAK) inhibitors targeting the type I interferon pathway. More selective interferon blockade with anifrolumab and next-generation B-cell depletion with chimeric antigen receptor T-cell (CAR-T) cell therapy represent emerging options for patients with refractory disease. Calcinosis remains without a standardized treatment procedure, and evidence-based management is limited by the lack of pediatric randomized controlled trials. Prospective, multicenter studies are needed to establish treatment algorithms and improve long-term outcomes in this rare disease.

Cite this article as: Aslan E, Kisla Ekinci RM, Torunoglu Z, Akay N, Kasapcopur O. Juvenile dermatomyositis: A review of the literature. ArchRheumatol. Published online July 31, 2026. doi: 10.5152/ArchRheumatol.2026.26552.