Current Treatment, Emerging Translational Therapies, and New Therapeutic Targets for Autoimmune Myasthenia Gravis.
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.
Neuromuscular Division, Department of Neurology, Duke University Medical Center, Durham, NC, USA.
Jeffrey T
JT
Guptill
Department of Neurological Sciences, Rush University Medical Center, Chicago, IL, USA.
Madhu
M
Soni
Department of Neurological Sciences, Rush University Medical Center, Chicago, IL, USA. matthew_meriggioli@rush.edu.
Matthew N
MN
Meriggioli
D001402:B-Lymphocytes / Q000187:drug effects / Q000502:physiology; D051056:Complement Inactivating Agents / Q000627:therapeutic use; D006801:Humans; D009157:Myasthenia Gravis / Q000188:drug therapy* / Q000276:immunology / Q000628:therapy; D010950:Plasma Cells / Q000187:drug effects / Q000502:physiology; D013601:T-Lymphocytes / Q000187:drug effects / Q000502:physiology; D057170:Translational Research, Biomedical
D051056:Complement Inactivating Agents
Autoimmune; Immunomodulation; Myasthenia gravis; Neuromuscular junction