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

Abstract

Introduction: 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: Dermatomyositis; IVIG; JAK/STAT; autoantibodies; inflammatory myopathies.

Figure 1.

Treatment mechanisms in Dermatomyositis. IL: Interleukin, TCR: T-Cell Receptor, TLR: Toll-like Receptor, MHC: Major Histocompatibility Complex, HCQ: Hydroxychloroquine, AZA: Azathioprine, MTX: Methotrexate, MMF: Mycophenolate, LEF: Leflunomide: CHLR: Chlorambucil. Not included in this figure: Adrenocorticotrophic Hormone Gel, Dapsone, Apremilast, Naltrexone, Lenabasum, Hematopoietic Stem Cell Transplantation