Immunotherapies for Immune-Mediated Myopathies: A Current Perspective

Abstract

Until recently, the treatment of immune-mediated inflammatory myopathies has largely been empirical with glucocorticoids, steroid-sparing immunosuppressive drugs, and intravenous immunoglobulin. However, a proportion of patients are only partially responsive to these therapies, and there has been a need to consider alternative treatment approaches. In particular, patients with inclusion body myositis are resistant to conventional immunotherapies or show only a transient response, and remain a major challenge. With increasing recognition of the different subtypes of immune-mediated inflammatory myopathies, and improved understanding of their pathogenesis, more targeted treatments are now being trialled. The overall approach to treatment, and novel therapies targeting B cells, T cells, and specific cytokines are discussed in this review.

Keywords: Immune-mediated myopathy; Inflammatory myopathy; Myositis; Treatment.

Fig. 1

Proposed schema for the use of currently available treatment modalities in immune-mediated inflammatory myopathies (IMIM). IV = intravenous; MTX = methotrexate; AZA = azathioprine; MMF = mycophenolate mofetil; ILD = interstitial lung disease; DM = dermatomyositis; NAM = necrotizing autoimmune myopathy