Immunosuppressive and immunomodulatory therapies for neuromuscular diseases. Part I: Traditional agents

Muscle Nerve. 2020 Jan;61(1):5-16. doi: 10.1002/mus.26708. Epub 2019 Nov 4.


Immunosuppressive and immunomodulatory therapies have had a major effect on the treatment of immune-mediated neuromuscular diseases. After the landmark introduction of synthetic corticosteroids, other therapies have become available, including plasma exchange (PLEX), immunoglobulin G (IgG), and steroid-sparing immunosuppressive drugs. More recently, novel biologically derived and antigen-specific pharmaceuticals have entered neuromuscular practice. Various levels of evidence guide the use of these treatments. This article reviews current immune-based therapies in neuromuscular diseases and is divided into two parts. Part I provides an update on the evidence and use of traditional therapies, such as corticosteroids, PLEX, intravenously delivered IgG (IVIG), and steroid-sparing immunosuppressive drugs. Part II focuses on the recently US Food and Drug Administration-approved therapies eculizumab and subcutaneous IgG (SCIG), the current indications for rituximab in neuromuscular disease, and on novel immunosuppressive therapeutic approaches under development.

Keywords: IVIG; azathioprine; corticosteroids; methotrexate; neuromuscular diseases; plasma exchange.

Publication types

  • Review
  • Comment

MeSH terms

  • Humans
  • Immunomodulation
  • Immunosuppressive Agents
  • Neuromuscular Diseases*
  • Plasma Exchange
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating*


  • Immunosuppressive Agents