Treatment principles in the management of autoimmune myasthenia gravis.
The pathogenesis of myasthenia gravis (MG) involves a T cell-directed antibody-mediated autoimmune attack on the nicotinic acetylcholine receptor (AChR) or, occasionally, on other postsynaptic antigens. The antibodies induce their effects through complement-mediated destruction of the postsynaptic endplate membrane with resultant reduction in endplate AChR, and to a lesser degree by increased turnover of endplate AChR or blockade of AChR function. Considerable progress in the treatment of MG has accrued from so-called symptomatic treatments, including improved critical care of seriously ill patients and medications (e.g., cholinesterase inhibitors) increasing the concentration of acetylcholine at the remaining endplate AChRs. Information from other autoimmune diseases and from the response of the normal immune system to invading pathogens supports the view that the course of MG is characterized by exacerbations and remissions. Therefore, the goal in MG treatment is to induce and maintain a remission. This usually involves combinations of short-term and long-term immunosuppressive agents. Selection of the particular combinations of agents in a given patient is guided by the goal of minimizing the cost/benefit ratio of the regimen in an individual patient. In general, the plan involves an initial forceful attack followed by a slow and measured withdrawal.
Richman
David P
DP
Department of Neurology, University of California, Davis, Davis, California 95616, USA. dprichman@ucdavis.edu
Agius
Mark A
MA
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000628:therapy*; D019468:Disease Management; D006801:Humans; D007116:Immunization, Passive; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009157:Myasthenia Gravis / Q000276:immunology / Q000628:therapy*; D011978:Receptors, Nicotinic / Q000276:immunology*; D013934:Thymectomy; D013997:Time Factors; D014180:Transplantation
D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D011978:Receptors, Nicotinic