IDIOPATHIC inflammatory myopathies are complex immune-mediated disorders with multisystem involvement, and recent advances are reshaping both classification and treatment approaches. Two-thirds of adults with these conditions experience a chronic disease course, underscoring the need for precise diagnosis and long-term care planning.
Autoantibodies now play a key role in defining disease subtypes and predicting associated complications, including cancer and interstitial lung disease. Their utility in identifying risk profiles has sharpened the ability of clinicians to tailor monitoring and intervention strategies. Multidisciplinary care remains essential, integrating rheumatology, neurology, pulmonology, dermatology, and oncology expertise to address the diverse clinical manifestations.
Therapeutically, intravenous immunoglobulins have demonstrated efficacy for patients with refractory dermatomyositis, improving both cutaneous and muscular disease activity. Beyond this established option, a wave of randomized controlled trials is advancing novel interventions. Central to these developments is a better understanding of disease mechanisms. In dermatomyositis, induction of interferons drives upregulation of interferon-stimulated genes across blood, skin, and muscle. These molecular signatures are emerging as both diagnostic biomarkers and measures of disease activity.
Therapeutic innovation is increasingly targeting these pathways. Approaches include blocking interferon activity through monoclonal autoantibodies, or intervening downstream with JAK inhibitors. Cellular immunotherapy also represents a promising frontier, with CD19-directed chimeric antigen receptor T-cell therapy under investigation for patients with refractory disease. Parallel research is exploring strategies to target T cells and specific T-cell subsets, expanding the arsenal of potential treatment modalities.
Together, these advances are positioning idiopathic inflammatory myopathies at the forefront of precision medicine in autoimmune disease. With biomarker-driven classification, established and emerging therapies, and a strong pipeline of clinical trials, the field is entering a new phase where outcomes may be improved through earlier identification and targeted intervention.
Reference:
Raaphorst J et al. Advances in the classification and management of idiopathic inflammatory myopathies. Lancet Neurol. 2025;24:776-788.
