Treatment of Myasthenia Gravis

吡啶斯替明 医学 重症肌无力 乙酰胆碱酯酶 硫唑嘌呤 药物治疗 临床试验 弱点 溴化吡啶斯替明 肌肉无力 药理学 疾病 内科学 外科 生物化学 化学
作者
Lorenzo Maggi,Renato Mantegazza
出处
期刊:Clinical Drug Investigation [Springer Nature]
卷期号:31 (10): 691-701 被引量:87
标识
DOI:10.2165/11593300-000000000-00000
摘要

Acquired myasthenia gravis (MG) is a chronic autoimmune disorder of the neuromuscular junction, characterized clinically by muscle weakness and abnormal fatigability on exertion. Current guidelines and recommendations for MG treatment are based largely on clinical experience, retrospective analyses and expert consensus. Available therapies include oral acetylcholinesterase (AChE) inhibitors for symptomatic treatment, and short- and long-term disease-modifying treatments. This review focuses on treatment of MG, mainly on the use of the AChE inhibitor pyridostigmine. Despite a lack of data from well controlled clinical trials to support their use, AChE inhibitors, of which pyridostigmine is the most commonly used, are recommended as first-line therapy for MG. Pyridostigmine has been used as a treatment for MG for over 50 years and is generally considered safe. It is suitable as a long-term treatment in patients with generalized non-progressive milder disease, and as an adjunctive therapy in patients with severe disease who are also receiving immunotherapy. Novel AChE inhibitors with oral antisense oligonucleotides have been developed and preliminary results appear to be promising. In general, however, AChE inhibitors provide only partial benefit and most patients eventually switch to long-term immunosuppressive therapies, most frequently corticosteroids and/or azathioprine. Although AChE inhibitors are known to be well tolerated and effective in relieving the symptoms of MG, further efforts are required to improve treatment options for the management of this disorder.
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