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重症肌无力:从自身抗体到治疗。

Myasthenia gravis: from autoantibodies to therapy.

机构信息

Neurology IV, Neuroimmunology and Neuromuscular Diseases Unit, Fondazione Istituto Neurologico 'Carlo Besta,' Milan, Italy.

出版信息

Curr Opin Neurol. 2018 Oct;31(5):517-525. doi: 10.1097/WCO.0000000000000596.

Abstract

PURPOSE OF REVIEW

The current article reviews the recent advances in the field of myasthenia gravis, which span from autoantibody profiling and pathogenic mechanisms to therapy innovation. The overview is highlighting specifically the data and the needs of targeted treatments in the light of precision medicine in myasthenia gravis.

RECENT FINDINGS

Novel data published recently further increased our knowledge on myasthenia gravis. The use of cell-based assays has greatly improved autoantibody detection in myasthenia gravis patients, and the mechanisms of action of these antibodies have been described. The role of Toll-like receptor activation in the generation of thymic alterations and anti-acetylcholine receptor autosensitization has been further investigated implementing our understanding on the relationships between innate immunity and autoimmunity. Additional studies have been focused on the alterations of T-cell/B-cell regulatory mechanisms in thymus and peripheral blood of myasthenia gravis patients. microRNAs and genetic factors are also emerging as key biomarkers in myasthenia gravis pathogenesis and prediction of drug efficacy in individual patients.

SUMMARY

The recent immunological and pathological findings in myasthenia gravis promise to improve myasthenia gravis treatment, via the development of more precise and personalized therapies.

摘要

目的综述

本文综述了重症肌无力领域的最新进展,涵盖了自身抗体谱和发病机制到治疗创新等多个方面。本文特别强调了精准医学背景下针对重症肌无力的靶向治疗的数据和需求。

最近发现

最近发表的新数据进一步增加了我们对重症肌无力的认识。基于细胞的检测方法极大地提高了重症肌无力患者自身抗体的检测水平,并描述了这些抗体的作用机制。通过实施我们对先天免疫和自身免疫之间关系的理解,进一步研究了 Toll 样受体激活在胸腺改变和抗乙酰胆碱受体自身致敏中的作用。其他研究集中在重症肌无力患者胸腺和外周血中 T 细胞/ B 细胞调节机制的改变上。microRNAs 和遗传因素也作为重症肌无力发病机制和预测个体患者药物疗效的关键生物标志物而出现。

总结

重症肌无力的最近免疫学和病理学发现有望通过开发更精确和个性化的治疗方法来改善重症肌无力的治疗。

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