Myasthenia gravis: a clinical-immunological update.
Myasthenia gravis: a clinical-immunological update.
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DOI：
10.1007/s00415-015-7963-5
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发表时间：
2016-04
影响因子：
6
通讯作者：
Palace J
中科院分区：
文献类型：
--
作者：
Binks S;Vincent A;Palace J
重症肌无力（MG）是神经肌肉连接处和自身抗体介导疾病的典型疾病。在大多数患者中，针对乙酰胆碱受体的igg1显性抗体会引起骨骼肌的疲劳性无力。在其余部分中，可变比例具有肌肉特异性酪氨酸激酶的抗体，而其余的血清阴性MG正在通过使用受体聚类技术的基于细胞的检测来解释，并且在较小程度上提出了新的抗原靶点。MG的发病率和流行率正在增加，特别是在老年人中。新的治疗方法正在开发中，胸腺切除术治疗非胸腺瘤性MG的随机对照试验结果将于2016年初公布，将具有特殊的临床价值。为了帮助导航不同质量的证据基础，执业临床医生可能会在妊娠、眼部和全身性MG （GMG）领域咨询新的MG指南。本文综述了成人GMG的流行病学、免疫学、治疗和临床方面的最新进展。
Myasthenia gravis (MG) is the archetypic disorder of both the neuromuscular junction and autoantibody-mediated disease. In most patients, IgG1-dominant antibodies to acetylcholine receptors cause fatigable weakness of skeletal muscles. In the rest, a variable proportion possesses antibodies to muscle-specific tyrosine kinase while the remainder of seronegative MG is being explained through cell-based assays using a receptor-clustering technique and, to a lesser extent, proposed new antigenic targets. The incidence and prevalence of MG are increasing, particularly in the elderly. New treatments are being developed, and results from the randomised controlled trial of thymectomy in non-thymomatous MG, due for release in early 2016, will be of particular clinical value. To help navigate an evidence base of varying quality, practising clinicians may consult new MG guidelines in the fields of pregnancy, ocular and generalised MG (GMG). This review focuses on updates in epidemiology, immunology, therapeutic and clinical aspects of GMG in adults.