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静脉注射免疫球蛋白在自身免疫性神经肌肉疾病中的应用

Intravenous immunoglobulin in autoimmune neuromuscular diseases.

作者信息

Dalakas Marinos C

机构信息

Neuromuscular Diseases Section, National Institutes of Health, National Institute of Neurological Disorders and Stroke, Bethesda, Md 20892, USA.

出版信息

JAMA. 2004 May 19;291(19):2367-75. doi: 10.1001/jama.291.19.2367.

Abstract

CONTEXT

Intravenous immunoglobulin (IVIG) enhances immune homeostasis by modulating expression and function of Fc receptors, interfering with activation of complement and production of cytokines, providing anti-idiotypic antibodies, and affecting the activation and effector functions of T and B cells. These mechanisms may explain the effectiveness of IVIG in autoimmune neuromuscular disorders.

OBJECTIVE

To systematically review the current status of the treatment of autoimmune neuromuscular diseases with IVIG, with emphasis on controlled trials.

DATA SOURCES

Peer-reviewed publications identified through MEDLINE (1966-2003), EMBASE (1974-2003), and references from bibliographies of pertinent articles. Each autoimmune neuromuscular disease term was searched in combination with the term intravenous immunoglobulin.

STUDY SELECTION AND DATA EXTRACTION

Criteria for selection of studies included controlled study design, English language, and clinical pertinence. Data quality was based on venue of publication and relevance to clinical care.

DATA SYNTHESIS

Outcomes of controlled trials indicate that IVIG at a total dose of 2 g/kg is effective as first-line therapy in Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy and as second-line therapy in stiff-person syndrome, dermatomyositis, myasthenia gravis, and Lambert-Eaton myasthenic syndrome. In other controlled studies, IVIG produced a modest, variable, and transient but not statistically significant benefit in patients with inclusion body myositis and paraproteinemic anti-myelin-associated glycoprotein antibody demyelinating polyneuropathy. Intravenous immunoglobulin is not effective in patients with multiple sclerosis who have established weakness or optic neuritis. In myasthenia gravis, it should be reserved for difficult cases or before thymectomy in lieu of plasma exchange.

CONCLUSION

Intravenous immunoglobulin is effective in many autoimmune neurologic diseases, but its spectrum of efficacy, especially as first-line therapy, and the appropriate dose for long-term maintenance therapy are not fully established. Further controlled studies of IVIG, combined with a dose-finding effect, pharmacoeconomics, and quality-of-life assessments, are warranted to improve the evidence base for clinical practice.

摘要

背景

静脉注射免疫球蛋白(IVIG)通过调节Fc受体的表达和功能、干扰补体激活和细胞因子产生、提供抗独特型抗体以及影响T和B细胞的激活及效应功能来增强免疫稳态。这些机制可能解释了IVIG在自身免疫性神经肌肉疾病中的有效性。

目的

系统回顾IVIG治疗自身免疫性神经肌肉疾病的现状,重点关注对照试验。

数据来源

通过MEDLINE(1966 - 2003年)、EMBASE(1974 - 2003年)以及相关文章参考文献中检索到的同行评审出版物。每个自身免疫性神经肌肉疾病术语都与“静脉注射免疫球蛋白”一词联合检索。

研究选择与数据提取

研究选择标准包括对照研究设计、英文发表以及临床相关性。数据质量基于发表渠道和与临床护理的相关性。

数据综合

对照试验结果表明,总剂量为2 g/kg的IVIG作为一线治疗在格林 - 巴利综合征、慢性炎症性脱髓鞘性多发性神经病和多灶性运动神经病中有效,作为二线治疗在僵人综合征、皮肌炎、重症肌无力和兰伯特 - 伊顿肌无力综合征中有效。在其他对照研究中,IVIG对包涵体肌炎和副蛋白血症性抗髓鞘相关糖蛋白抗体脱髓鞘性多发性神经病患者产生了适度、可变且短暂但无统计学显著意义的益处。静脉注射免疫球蛋白对已出现肌无力或视神经炎的多发性硬化症患者无效。在重症肌无力中,应将其保留用于难治性病例或胸腺切除术前代替血浆置换。

结论

静脉注射免疫球蛋白在许多自身免疫性神经疾病中有效,但其疗效范围,尤其是作为一线治疗的疗效范围,以及长期维持治疗的合适剂量尚未完全确定。有必要对IVIG进行进一步的对照研究,并结合剂量探索效应、药物经济学和生活质量评估,以改善临床实践的证据基础。

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