Abstract
BACKGROUND: Juvenile dermatomyositis (JDM), the most common inflammatory myopathy of childhood, is a complex autoimmune condition with significant disease burden and variable treatment responses. While conventional immunosuppression remains standard, advances in immunopathology are revealing new therapeutic and biomarker opportunities. MAIN BODY: This review provides emerging insights into JDM pathogenesis. Interferon (IFN)-stimulated gene (ISG) expression, and IFN-related emerging biomarkers which may be valuable for monitoring disease activity and treatment response. Mitochondrial dysfunction, involving oxidative stress and oxidized mtDNA, contributes to systemic pathology and offers new therapeutic targets. Other critical pathogenic processes include pyroptosis, neutrophil extracellular traps, and abnormal B-cell activity. Novel treatments targeting IFN pathways, B cells, and mitochondrial oxidation are under investigation. CAR T-cell therapies have shown early promise in refractory cases. CONCLUSION: Ongoing research is transforming our understanding of JDM, enabling biomarker-driven precision medicine and targeted therapies. These advances offer the potential to improve outcomes and achieve durable remission, even in refractory or high-risk disease subsets.