Chinese Journal of Contemporary Neurology and Neurosurgery ›› 2020, Vol. 20 ›› Issue (1): 48-54. doi: 10.3969/j.issn.1672-6731.2020.01.008

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Progress in rituximab for anti-MuSK antibody-related myasthenia gravis

LI Ting, YANG Li   

  1. Department of Neurology, Tianjin Medical University General Hospital, Tianjin 300052, China
  • Received:2019-12-28 Online:2020-01-25 Published:2020-01-21
  • Supported by:

    This study was supported by the National Natural Science Foundation of China (No. 81771363).

利妥昔单抗在抗MuSK抗体阳性重症肌无力中的应用进展

李婷, 杨丽   

  1. 300052 天津医科大学总医院神经内科
  • 通讯作者: 杨丽,Email:yangli2001@tmu.edu.cn
  • 基金资助:

    国家自然科学基金资助项目(项目编号:81771363)

Abstract:

Myasthenia gravis (MG) is an autoantibody mediated autoimmune disease of the neuromuscular junction (NMJ), and anti-muscle-specific receptor tyrosine kinase (MuSK) antibody-related MG (MuSK-MG) covers 5%-8% of all MG patients. MuSK-MG is a life threatening condition, characterized with early bulbar, neck, and respiratory muscle weakness. MuSK-MG patients have poor response to standard therapies, known as drug-resistant patients. Rituximab (RTX) is a monoclonal antibody that targets the CD20 antigen on B cells. At present, most studies have described clinical improvement and immunosuppressant dose reduction following RTX treatment of refractory MG patients, especially for MuSK-MG patients.

Key words: Myasthenia gravis, Muscle-specific receptor tyrosine kinase (not in MeSH), Rituximab, Review

摘要:

重症肌无力是一种自身抗体介导的针对神经肌肉接头突触后膜的自身免疫性疾病,抗肌肉特异性受体酪氨酸激酶(MuSK)抗体阳性(MuSK-MG)患者占所有重症肌无力患者的5%~8%。与抗乙酰胆碱受体(AChR)抗体阳性和血清抗AChR和MuSK抗体阴性患者相比,MuSK-MG患者发病时间早、病情严重、应用传统治疗不能有效控制疾病进展。利妥昔单抗是通过基因重组技术研制的针对B细胞表面CD20抗原的人鼠嵌合型单克隆抗体,可以有效治疗难治性重症肌无力,减少复发和肌无力危象次数,对难治性MuSK-MG患者具有良好的有效性和安全性。

关键词: 重症肌无力, 肌肉特异性受体酪氨酸激酶(非MeSH词), 利妥昔单抗, 综述