[Therapeutic strategy in inflammatory myopathies (polymyositis, dermatomyositis, overlap myositis, and immune-mediated necrotizing myopathy)]

Abstract

Inflammatory myopathies (IM) are a heterogeneous group of autoimmune muscle disorders of unknown origin that share clinical symptoms such as muscle weakness and histological features with the presence in muscle of inflammatory infiltrate. Based on clinical, histological and serological characteristics, IM can be divided into polymyositis, dermatomyositis, overlap myositis, cancer-associated myositis, immune-mediated necrotizing myopathy, and inclusion-body myositis. Because of their resistance to corticosteroids and immunosuppressive drugs, inclusion-body myositis will be treated separately in this issue. Major obstacles in conducting high quality randomized controlled trials in inflammatory myopathies include the low prevalence and the heterogeneity of these diseases as well as the lack of international consensus on the outcome measures. In the absence of adequate controlled therapeutic trials, treatment of these disorders remains largely empirical. Corticosteroids are the cornerstone therapy. Due to the chronic course of the disease, there is a frequent need to use additional immunosuppressive treatment both to improve the disease response and to reduce the side effects of corticosteroids. Intravenous immunoglobulin infusion is a costly treatment option that is reserved in the presence of refractory dermatomyositis based on a trial showing superior efficacy against control in patients with impaired swallowing or with contraindications to immunosuppressive drugs. In patients who fail second-line therapy, which usually consists of methotrexate plus corticosteroids, the diagnosis should be carefully reassessed before considering other treatment options including methotrexate plus azathioprine or biological agents such as rituximab.

Keywords: Dermatomyosite; Dermatomyositis; Immune-mediated necrotizing myopathy; Inflammatory myopathy; Myopathie inflammatoire; Myopathie nécrosante auto-immune; Polymyosite; Polymyositis; Traitement; Treatment.