![]() Autoreactive T cells that escape this process or arise de novo, are kept in check in the peripheral circulation by a subset of CD4+ cells called Treg cells that bring about apoptosis, anergy or suppression of autoreactive cells ( 3). The normal immune system weeds out autoreactive T cells early and these are destroyed in the thymus by the process called central tolerance. MG is an antibody mediated disease in which the immunopathogenesis is T cell driven and there exists a complex interplay between CD4+ T cells and B cells. Overview of Myasthenia Gravis Pathogenesis These treatments usher in a new era of more focused MG management that promises to improve the lives of people with MG. These new treatments may help reduce the use of steroids, and their relatively fast onset of action makes them attractive options to traditional steroid-sparing agents. This review discusses novel agents that act on other nodal points in MG pathogenesis, autologous stem cell, and chimeric antigen receptor T (CART-T) cell therapy in MG. FC receptor inhibitors reduce the level of circulating pathogenic autoantibody, whereas terminal complement C5 inhibitors block the formation of the membrane attack complex at the last step of immune injury. These treatments work at different points of the immune pathology and are likely to be complementary in action. These include terminal complement C5 inhibitors and Fc receptor inhibitors ( 2). Recently more directed, novel immunotherapies have been developed. ![]() Traditional steroid-sparing agents have shown mixed efficacy in trials, and usually take several months to be effective. Corticosteroids are effective in a majority of MG patients however, these are associated with many long-term side effects, often intolerable for patients. Broad-based immunotherapies, such as corticosteroids, azathioprine, mycophenolate, tacrolimus, and cyclosporine, have been effective in controlling symptoms of myasthenia ( 1). Myasthenia gravis (MG) is the prototypical autoimmune disorder caused by specific autoantibodies at the neuromuscular junction. Most of these new agents have advantages over conventional immunosuppressive treatment (IST) for MG therapy in terms of faster onset of action, favourable side effect profile and the potential for a sustained and long-term remission. These agents include terminal complement C5 inhibitors, Fc receptor inhibitors, B cell depleting agents (anti CD 19 and 20 and B cell activating factor inhibitors), proteosome inhibitors, T cells and cytokine based therapies (chimeric antigen receptor T cell therapy), autologous stem cell transplantation, and subcutaneous immunoglobulin (SCIG). This review focuses on the aberrant immunological processes in MG, the novel agents that target them along with the clinical evidence for efficacy and safety. With advances in translational research and drug development capabilities, more directed therapeutic agents that can alter the future of MG treatment have been developed. While being effective in a majority of MG patients many of these immunosuppressive agents are associated with long-term side effects, often intolerable for patients, and take several months to be effective. ![]() ![]() Broad-based immunotherapies, such as corticosteroids, azathioprine, mycophenolate, tacrolimus, and cyclosporine, have been effective in controlling symptoms of myasthenia. Ellen & Martin Prosserman Centre for Neuromuscular Diseases, University Health Network, University of Toronto, Toronto, ON, Canada.Deepak Menon Carolina Barnett Vera Bril *
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