Current Treatment, Emerging Translational Therapies, and New Therapeutic Targets for Autoimmune Myasthenia Gravis

Neurotherapeutics. 2016 Jan;13(1):118-31. doi: 10.1007/s13311-015-0398-y.

Abstract

Myasthenia gravis (MG) is an autoimmune disease associated with the production of autoantibodies against 1) the skeletal muscle acetylcholine receptor; 2) muscle-specific kinase, a receptor tyrosine kinase critical for the maintenance of neuromuscular synapses; 3) low-density lipoprotein receptor-related protein 4, an important molecular binding partner for muscle-specific kinase; and 4) other muscle endplate proteins. In addition to the profile of autoantibodies, MG may be classified according the location of the affected muscles (ocular vs generalized), the age of symptom onset, and the nature of thymic pathology. Immunopathologic events leading to the production of autoantibodies differ in the various disease subtypes. Advances in our knowledge of the immunopathogenesis of the subtypes of MG will allow for directed utilization of the ever-growing repertoire of therapeutic agents that target distinct nodes in the immune pathway relevant to the initiation and maintenance of autoimmune disease. In this review, we examine the pathogenesis of MG subtypes, current treatment options, and emerging new treatments and therapeutic targets.

Keywords: Autoimmune; Immunomodulation; Myasthenia gravis; Neuromuscular junction.

Publication types

  • Review

MeSH terms

  • B-Lymphocytes / drug effects
  • B-Lymphocytes / physiology
  • Complement Inactivating Agents / therapeutic use
  • Humans
  • Myasthenia Gravis / drug therapy*
  • Myasthenia Gravis / immunology
  • Myasthenia Gravis / therapy
  • Plasma Cells / drug effects
  • Plasma Cells / physiology
  • T-Lymphocytes / drug effects
  • T-Lymphocytes / physiology
  • Translational Medical Research

Substances

  • Complement Inactivating Agents