Immunotherapies for Immune-Mediated Myopathies: A Current Perspective

Abstract

Summary 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.

Medical resource utilization in dermatomyositis/polymyositis patients treated with repository corticotropin injection, intravenous immunoglobulin, and/or rituximab

“…Propensity score matching was performed using a greedy propensity score algorithm without replacement 24. RCI patients were matched with other patients by five digits of propensity score.…”

Infections and the relationship to treatment in neuromuscular autoimmunity

“…One of the most important considerations of immunosuppressive drug therapy is the increased risk of serious or life-threatening infections. [1][2][3] Earlier studies in inflammatory muscle disease have shown that 27%-37% of patients acquire significant infections, many of which are caused by opportunistic pathogens. 4,5 Furthermore, these studies demonstrated that increased risk of infection was associated with various immunomodulatory treatments, including corticosteroids, azathioprine, and immunoglobulin therapies.…”

“…The mainstay of treatment for autoimmune neuromuscular disorders is immunotherapy aimed at modulating or reducing disease activity. One of the most important considerations of immunosuppressive drug therapy is the increased risk of serious or life‐threatening infections . Earlier studies in inflammatory muscle disease have shown that 27%–37% of patients acquire significant infections, many of which are caused by opportunistic pathogens .…”

Necrotizing myositis in a rectus muscle arising in the setting of long-standing Langerhans cell histiocystosis and recent dabrafenib treatment

“…Previous reports have described biopsy findings of necrotic myocytes with minimal surrounding infiltration, acute lymphocytic infiltrate, 20 or mixed inflammatory infiltrate, often macrophage-predominant with some T cells. 21 , 22 , 23 …”