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重症肌无力理解与管理方面的最新进展

Recent advances in understanding and managing myasthenia gravis.

作者信息

Jordan Allison, Freimer Miriam

机构信息

Department of Neurology, The Ohio State Wexner Medical Center, Columbus, Ohio, USA.

出版信息

F1000Res. 2018 Oct 31;7. doi: 10.12688/f1000research.15973.1. eCollection 2018.

Abstract

Autoimmune myasthenia gravis (MG) is a neuromuscular junction disorder marked clinically by fatigable muscle weakness and serologically by the presence of autoantibodies against acetylcholine receptors (AChRs), muscle-specific kinase (MuSK), or lipoprotein-related protein 4 (LPR4). Over the past few decades, the mortality of patients with MG has seen a dramatic decline secondary to evolving interventions in critical care and medical management. In the past 2 to 3 years, there have been several changes in standard of care for the treatment of MG. These changes include confirmation of the benefit of thymectomy versus medical management alone in AChR patients and a new US Food and Drug Administration-approved medication for refractory MG. There are also several exciting new prospective drugs in the pipeline, which are in different stages of clinical trial testing.

摘要

自身免疫性重症肌无力(MG)是一种神经肌肉接头疾病,临床特征为易疲劳性肌无力,血清学特征为存在针对乙酰胆碱受体(AChR)、肌肉特异性激酶(MuSK)或脂蛋白相关蛋白4(LPR4)的自身抗体。在过去几十年中,由于重症监护和药物治疗的不断发展,MG患者的死亡率大幅下降。在过去两到三年里,MG治疗的护理标准发生了一些变化。这些变化包括证实了在AChR患者中行胸腺切除术相对于单纯药物治疗的益处,以及一种新的美国食品药品监督管理局批准的用于难治性MG的药物。此外,还有几种令人兴奋的新型前瞻性药物正在研发中,处于不同阶段的临床试验测试。

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