Department of Neuromuscular Diseases and Neuroimmunology, Fondazione Istituto Neurologico Carlo Besta, Milan, Italy.
Neuropsychiatr Dis Treat. 2011;7:151-60. doi: 10.2147/NDT.S8915. Epub 2011 Mar 22.
Myasthenia gravis (MG) is an autoimmmune disease in which autoantibodies to different antigens of the neuromuscular junction cause the typical weakness and fatigability. Treatment includes anticholinesterase drugs, immunosuppression, immunomodulation, and thymectomy. The autoimmune response is maintained under control by corticosteroids frequently associated with immunosuppressive drugs, with improvement in the majority of patients. In case of acute exacerbations with bulbar symptoms or repeated relapses, modulation of autoantibody activity by plasmapheresis or intravenous immunoglobulins provides rapid improvement. Recently, techniques removing only circulating immunoglobulins have been developed for the chronic management of treatment-resistant patients. The rationale for thymectomy relies on the central role of the thymus. Despite the lack of controlled studies, thymectomy is recommended as an option to improve the clinical outcome or promote complete remission. New videothoracoscopic techniques have been developed to offer the maximal surgical approach with the minimal invasiveness and hence patient tolerability. The use of biological drugs such as anti-CD20 antibodies is still limited but promising. Studies performed in the animal model of MG demonstrated that several more selective or antigen-specific approaches, ranging from mucosal tolerization to inhibition of complement activity or cellular therapy, might be feasible. Investigation of the transfer of these therapeutic approaches to the human disease will be the challenge for the future.
重症肌无力（MG）是一种自身免疫性疾病，其中针对神经肌肉接头不同抗原的自身抗体导致典型的无力和易疲劳。治疗包括抗胆碱酯酶药物、免疫抑制、免疫调节和胸腺切除术。大多数患者通过经常与免疫抑制剂联合使用的皮质类固醇来控制自身免疫反应，从而改善病情。在出现延髓症状的急性恶化或反复复发的情况下，通过血浆置换或静脉注射免疫球蛋白来调节自身抗体活性可迅速改善病情。最近，为了治疗对治疗有抗性的患者，已经开发出仅去除循环免疫球蛋白的技术来进行慢性管理。胸腺切除术的基本原理是基于胸腺的核心作用。尽管缺乏对照研究，但胸腺切除术被推荐作为改善临床结果或促进完全缓解的一种选择。已经开发出新型的胸腔镜技术，以提供最小侵袭性和患者耐受性的最大手术方法。尽管抗 CD20 抗体等生物药物的使用仍然有限，但具有很大的应用前景。在 MG 的动物模型中进行的研究表明，从黏膜耐受到抑制补体活性或细胞治疗等更具选择性或抗原特异性的方法可能是可行的。将这些治疗方法应用于人类疾病的研究将是未来的挑战。
