Immunosuppressive and Immunomodulatory Therapies for Neuromuscular Diseases Part I: Traditional Agents

Authors:  Constantine Farmakidis, MD, Mazen Dimachkie, MD, Mamatha Pasnoor, MD, Richard J., Barohn, MD, Neurology Department, The University of Kansas Medical Center, Kansas City, KS, USA.

Published:  doi: 10.1002/mus.26708

Abstract: Immunosuppressive and immunomodulatory therapies have had a major effect on the treatment of immune-mediated neuromuscular diseases. Following 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 medications. This article reviews current immune-based therapies in neuromuscular diseases and is divided in 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 FDA-approved therapies eculizumab and subcutaneous IgG (SCIG), the current indications for rituximab in neuromuscular disease, and on novel immunosuppressive therapeutic approaches underdevelopment.