Emerging therapies in idiopathic inflammatory myopathies: Advances in treatment and future directions

Idiopathic inflammatory myopathies are a group of rare autoimmune diseases characterized by chronic muscle inflammation and variable systemic involvement. Although conventional therapies, including corticosteroids and immunosuppressants, form the cornerstone of treatment, most patients do not achieve remission, underscoring the need for more effective options. Advances in understanding disease pathogenesis have led to the development of novel targeted therapies. Strategies aimed at disrupting key immune pathways, such as interferon signaling, T cell activation, and B cell maturation, have shown promising results. Additionally, innovative approaches such as engineered cellular therapies are currently explored. These treatments offer the potential to achieve deeper and more durable disease control, reduce corticosteroid dependence, and improve quality of life for the patients. In this chapter, we present the recent findings on novel therapies for the treatment of IIM (all subtypes except IBM) by discussing the rationale behind their use, their potential role in improving patient outcomes in the different IIM subtypes, as well as the limitations of their wide implementation. Future strategies and promising therapies under investigation are also highlighted.