静脉注射免疫球蛋白在Stiff‐Person综合征的治疗和转归中的作用:一项系统综述

Q4 Immunology and Microbiology
Roshan Aryal, Suraj Shrestha, Sushan Homagain, Sansar Babu Tiwari, Rajeev Ojha
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引用次数: 0

摘要

僵直人综合征(SPS)是一种罕见的免疫介导的神经肌肉疾病,其特征是轴向和四肢肌肉僵硬、僵硬和间歇性痉挛。包括静脉注射免疫球蛋白(IVIG)在内的各种免疫疗法已被用于这种自身免疫性疾病。在这里,我们的目的是回顾IVIG在治疗SPS及其结果中的作用。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Intravenous immunoglobulin in the management and outcome of Stiff-Person syndrome: A systematic review

Background

Stiff Person Syndrome (SPS), a rare immune-mediated neuromuscular disorder, is characterized by rigidity, stiffness, and intermittent spasms of axial and extremity muscles. Various immunotherapies including intravenous immunoglobulin (IVIG) have been used for this autoimmune condition. Here we aim to review the role of IVIG in the treatment of SPS along with its outcome.

Methods

A systematic literature search of PubMed and Embase was conducted to identify the relevant published articles against the predefined criteria using suitable keywords combinations till September 20, 2021. Data were extracted to produce descriptive information of SPS patients on demographics, diagnostics, treatment with IVIG, and outcome.

Results

Twelve studies with 216 patients were included in the review and 63.89% of them had classical SPS. Glutamic acid decarboxylase (GAD) autoantibodies were present in 72.68% of the patients and 57.89% in whom electromyography (EMG) was performed had continuous motor activity. IVIG therapy was given to 95 patients in different regimens in various studies and varying scoring systems were used to assess the outcome, and 83.16% showed some form of improvement, 14.74% showed no improvement, while 2.10% worsened. None of the included studies mentioned an adverse effect of IVIG in the patients.

Conclusion

IVIG may benefit patients with SPS along with other medications. Owing to the rarity of the disease and insufficient studies on the assessment of immunotherapy in SPS, longitudinal studies with a sizable number of patients are required to clarify clinical course, treatment, and outcome in SPS with the use of IVIG.

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来源期刊
Clinical and Experimental Neuroimmunology
Clinical and Experimental Neuroimmunology Immunology and Microbiology-Immunology and Microbiology (miscellaneous)
CiteScore
1.60
自引率
0.00%
发文量
52
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