A comprehensive review of dermatomyositis treatments - from rediscovered classics to promising horizons

ABSTRACTIntroduction Dermatomyositis (DM) is a rare inflammatory disease with diverse cutaneous and systemic manifestations, often associated with myositis-specific antibodies. Managing patients with refractory DM, or individuals presenting pecific complications, like calcinosis or rapidly progressive interstitial lung disease, presents unique challenges.Areas covered This review explores current and promising treatment options for DM, drawing from clinical studies, case series, and case reports that consider the underlying disease pathophysiology.Expert opinion Recent advancements have improved our understanding and management of DM. The discovery of distinct DM autoantibodies and their correlation with specific clinical phenotypes has transformed patient categorization and enhanced our knowledge of the pathogenesis of the disease. Intravenous immunoglobulin, a well-established treatment in dermatomyositis, has regained prominence and a large randomized clinical trial has reaffirmed its efficacy, confirming it as an effective therapeutic option in this group of patients. Identification of the type I interferon pathway as a key pathogenic mechanism in DM has opened up new avenues for more effective treatment strategies. Blocking the JAK/STAT pathway offers potential for improved management of refractory patients and prevention of highly morbid complications. These recent advancements have significantly impacted the management and care of dermatomyositis patients, enabling tailored approaches, targeted interventions, and improved outcomes for individuals affected by this complex condition.KEYWORDS: Dermatomyositisinflammatory myopathiesJAK/STATIVIGautoantibodies Article highlights The treatment of dermatomyositis poses challenges that require tailored approaches, taking into account the risk of specific complications, comorbidities, and careful consideration of risk-benefit factors.Recent advancements in the therapeutic modulation of the JAK/STAT pathway have emerged as a valuable tool for managing refractory manifestations of DM. Furthermore, this approach holds promise in preventing highly severe manifestations of the disease, such as rapidly progressive interstitial lung disease (RP-ILD).The efficacy of intravenous immunoglobulin in the treatment of DM has been further supported, confirming its effectiveness in improving patient outcomes.Declaration of interestsThe authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.Reviewer disclosuresPeer reviewers on this manuscript have no relevant financial or other relationships to disclose.Additional informationFundingThis study was funded, in part, by the Intramural Research Program of the National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health.