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利妥昔单抗有效早期治疗乙酰胆碱受体抗体阳性重症肌无力:来自一个发展中国家神经免疫学诊所的经验

Effective Early Treatment of AChR Antibody-Positive Myasthenia Gravis with Rituximab; the Experience from a Neuroimmunology Clinic in a Developing Country.

作者信息

Mathew Thomas, Thomas Kurian, K John Saji, Venkatesh Shruthi, Nadig Raghunandan, Badachi Sagar, Souza Delon D, Sarma Grk, Parry Gareth J

机构信息

Department of Neurology, St. John's Medical College Hospital, Bengaluru, Karnataka, India.

出版信息

J Cent Nerv Syst Dis. 2021 May 17;13:11795735211016080. doi: 10.1177/11795735211016080. eCollection 2021.

DOI:10.1177/11795735211016080
PMID:34045914
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8135199/
Abstract

BACKGROUND

Rituximab is reserved for treating refractory myasthenia gravis (MG) patients. Here we report our experience with rituximab in AChR antibody positive generalized MG (gMG) and impending myasthenic crisis (IMC).

METHODS

This retrospective, observational study, conducted at a tertiary care, neuroimmunology clinic, analyzed the data of patients with AChR antibody positive gMG, treated with rituximab between 1st January 2016 and 30th October 2018.

RESULTS

Eleven patients with AChR antibody positive gMG received rituximab. Mean age of the cohort was 50.54 ± 18.71 years with 9 males. Seven out of 11 patients received rituximab in the early stage (<2 years from onset) and had good response to treatment. Four of the 5 patients with IMC improved with rituximab alone. In the 10 patients who regularly followed up, there was a significant difference between the QMG scores at baseline and at 1, 2, 6, 12, and 18 months ( < .0001).

CONCLUSION

Rituximab appears to be a potentially effective early treatment option for AChR antibody positive generalized MG and impending myasthenic crisis.

摘要

背景

利妥昔单抗 reserved 用于治疗难治性重症肌无力(MG)患者。在此,我们报告我们在抗乙酰胆碱受体(AChR)抗体阳性的全身型 MG(gMG)和重症肌无力危象(IMC)中使用利妥昔单抗的经验。

方法

这项回顾性观察性研究在一家三级医疗神经免疫学诊所进行,分析了2016年1月1日至2018年10月30日期间接受利妥昔单抗治疗的AChR抗体阳性gMG患者的数据。

结果

11例AChR抗体阳性gMG患者接受了利妥昔单抗治疗。该队列的平均年龄为50.54±18.71岁,男性9例。11例患者中有7例在疾病早期(发病<2年)接受了利妥昔单抗治疗,且对治疗反应良好。5例IMC患者中有4例仅使用利妥昔单抗后病情改善。在10例定期随访的患者中,基线时与1、2、6、12和18个月时的重症肌无力定量评分(QMG)之间存在显著差异(<0.0001)。

结论

利妥昔单抗似乎是抗AChR抗体阳性全身型MG和重症肌无力危象一种潜在有效的早期治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/96ff/8135199/92f177a91319/10.1177_11795735211016080-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/96ff/8135199/054e3969e36d/10.1177_11795735211016080-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/96ff/8135199/accd0e0992bc/10.1177_11795735211016080-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/96ff/8135199/92f177a91319/10.1177_11795735211016080-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/96ff/8135199/054e3969e36d/10.1177_11795735211016080-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/96ff/8135199/accd0e0992bc/10.1177_11795735211016080-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/96ff/8135199/92f177a91319/10.1177_11795735211016080-fig3.jpg

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JAMA Neurol. 2020 Aug 1;77(8):974-981. doi: 10.1001/jamaneurol.2020.0851.
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Rituximab in AChR subtype of myasthenia gravis: systematic review.
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Aging Dis. 2023 Aug 1;14(4):1070-1092. doi: 10.14336/AD.2022.1215.
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