单位:[1]Department of Neurology, George Washington University, Washington DC 20008[2]Department of Neuroscience, Clinical Neurophysiology, Uppsala University, Uppsala, Sweden[3]Department of Neurology, Duke University Medical Center, Durham, North Carolina, USA
Introduction: Myasthenia gravis (MG) is an autoimmune neuromuscular disorder with no cure and conventional treatments limited by significant adverse effects and variable benefit. In the last decade, therapeutic development has expanded based on improved understanding of autoimmunity and financial incentives for drug development in rare disease. Clinical subtypes exist based on age, gender, thymic pathology, autoantibody profile, and other poorly defined factors, such as genetics, complicate development of specific therapies. Areas covered: Clinical presentation and pathology vary considerably among patients with some having weakness limited to the ocular muscles and others having profound generalized weakness leading to respiratory insufficiency. MG is an antibody-mediated disorder dependent on autoreactive B cells which require T-cell support. Treatments focus on elimination of circulating autoantibodies or inhibition of effector mechanisms by a broad spectrum of approaches from plasmapheresis to B-cell elimination to complement inhibition. Expert commentary: Standard therapies and those under development are disease modifying and not curative. As a rare disease, clinical trials are challenged in patient recruitment. The great interest in development of treatments specific for MG is welcome, but decisions will need to be made to focus on those that offer significant benefits to patients.
基金:
Muscular Dystrophy AssociationMuscular Dystrophy Association [508240]; National Institutes of HealthUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USA [K23NS085049, HHSN27100001]; NATIONAL EYE INSTITUTEUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Eye Institute (NEI) [R24EY014837] Funding Source: NIH RePORTER; NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKEUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute of Neurological Disorders & Stroke (NINDS) [K23NS085049] Funding Source: NIH RePORTER
第一作者单位:[1]Department of Neurology, George Washington University, Washington DC 20008
通讯作者:
通讯机构:[1]Department of Neurology, George Washington University, Washington DC 20008[*1]Department of Neurology,GW-Medical Faculty Associates,2150 Pennsylvania Avenue,Washington DC, 20008 USA
推荐引用方式(GB/T 7714):
Shuhui Wang,Iva Breskovska,Shreya Gandhy,et al.Advances in autoimmune myasthenia gravis management[J].EXPERT REVIEW of NEUROTHERAPEUTICS.2018,18(7):573-588.doi:10.1080/14737175.2018.1491310.
APA:
Shuhui Wang,Iva Breskovska,Shreya Gandhy,Anna Rostedt Punga,Jeffery T. Guptill&Henry J. Kaminski.(2018).Advances in autoimmune myasthenia gravis management.EXPERT REVIEW of NEUROTHERAPEUTICS,18,(7)
MLA:
Shuhui Wang,et al."Advances in autoimmune myasthenia gravis management".EXPERT REVIEW of NEUROTHERAPEUTICS 18..7(2018):573-588