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[重症肌无力的治疗策略]

[Therapeutic strategy in myasthenia gravis].

作者信息

Tranchant C

机构信息

Service de Neurologie, Hôpitaux Universitaires, Hôpital Civil, 67091 Strasbourg Cedex.

出版信息

Rev Neurol (Paris). 2009 Feb;165(2):149-54. doi: 10.1016/j.neurol.2009.01.005.

Abstract

The purpose of the treatment of autoimmune myasthenia gravis is to directly improve neuromuscular transmission, and also to reduce the production or presence of the nicotinic acetylcholine receptor (achR). Acetylcholinesterase inhibitors are the first line treatment with the rapid onset of effect, for all types of myasthenia gravis (ocular, generalized myasthenia gravis, seronegative or seropositive patients). Plasmapheresis or intravenous immunoglobulin (IVIg) is the treatment for exacerbations. Their main advantage is the rapid onset of the effect. Three to five plasma exchanges or IVIg infusions (1.2 to 2 g/b.w administered over 2-5 days) are usually recommended. In case of suspected thymoma, thymectomy should be always performed. The option of thymectomy is discussed in young patients less than 50 years old with unstable myasthenia gravis, even if thymoma lesions are not suspected. Corticosteroids and/or immunosuppressive agents are used in severe forms of the disease. A few randomized studies have shown the efficacy of the therapeutic agents. Corticosteroids are considered a major treatment of myasthenia gravis but the doses and periods of time are still being debated. The combination of corticosteroids and immunosuppressive agents are recommended early to spare corticosteroids. The treatment of myasthenia gravis should be modulated regularly (minimal doses for example).

摘要

自身免疫性重症肌无力的治疗目的是直接改善神经肌肉传递,同时减少烟碱型乙酰胆碱受体(achR)的产生或存在。乙酰胆碱酯酶抑制剂是所有类型重症肌无力(眼肌型、全身型重症肌无力、血清阴性或血清阳性患者)的一线治疗药物,起效迅速。血浆置换或静脉注射免疫球蛋白(IVIg)用于治疗病情加重。它们的主要优点是起效快。通常建议进行三到五次血浆置换或IVIg输注(1.2至2 g/体重,在2至5天内给药)。如果怀疑有胸腺瘤,应始终进行胸腺切除术。对于年龄小于50岁、重症肌无力病情不稳定的年轻患者,即使未怀疑有胸腺瘤病变,也会讨论胸腺切除术的选择。对于病情严重的患者,使用皮质类固醇和/或免疫抑制剂。一些随机研究表明了这些治疗药物的疗效。皮质类固醇被认为是重症肌无力的主要治疗药物,但剂量和疗程仍在争论中。建议早期联合使用皮质类固醇和免疫抑制剂,以减少皮质类固醇的用量。重症肌无力的治疗应定期调整(例如使用最小剂量)。

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