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静脉注射免疫球蛋白与血浆置换治疗重症肌无力病情恶化:证据何在?:一项严格评价主题

IVIG Versus PLEX in the Treatment of Worsening Myasthenia Gravis: What is the Evidence?: A Critically Appraised Topic.

作者信息

Dhawan Priya S, Goodman Brent P, Harper Charles M, Bosch Peter E, Hoffman-Snyder Charlene R, Wellik Kay E, Wingerchuk Dean M, Demaerschalk Bart M

机构信息

*Department of Neurology, Mayo Clinic ‡Department of Library service, Division of Education Administration, Mayo Clinic, Scottsdale §Department of Neurology, Mayo Clinic, Phoenix, AZ †Department of Neurology, Mayo Clinic, Rochester, NY.

出版信息

Neurologist. 2015 May;19(5):145-8. doi: 10.1097/NRL.0000000000000026.

Abstract

BACKGROUND

Immune therapies such as intravenous immunoglobulin (IVIG) and plasma exchange (PLEX) are first line in the treatment of worsening myasthenia gravis. Although PLEX is favored in myasthenic crisis, IVIG is increasingly used in exacerbations due to cost and ease of administration.

OBJECTIVES

To review and critically assess current evidence on the effects of IVIG and PLEX on functional outcomes in patients with worsening myasthenia gravis.

METHODS

A structured critical appraisal was conducted on the objective topic. This included a creation of a structured question based on a clinical scenario, comprehensive literature search, selection of evidence for review, and critical appraisal of selected evidence. Evidence was summarized and commentary provided. Participants included consultant and resident neurologists, a medical librarian, clinical epidemiologists, and content experts in the field of neuromuscular neurology.

RESULTS

A single-blinded, randomized-controlled trial that compared IVIG and PLEX in 84 patients with worsening myasthenia gravis was selected for review. Primary outcome measure was functional status at 14 days after treatment, as assessed by the Quantitative Myasthenia Gravis Score. Change in Quantitative Myasthenia Gravis Score at day 14 for all subjects was 4.0, without statistically significant differences between IVIG and PLEX groups.

CONCLUSIONS

IVIG and PLEX are equally effective in worsening myasthenia gravis. Treatment decisions may depend on several variables, including presence of respiratory distress, medical comorbidities, access to medication, and cost. PLEX will likely remain the treatment of choice in true myasthenic crisis.

摘要

背景

静脉注射免疫球蛋白(IVIG)和血浆置换(PLEX)等免疫疗法是治疗重症肌无力病情恶化的一线疗法。尽管在重症肌无力危象中更倾向于使用血浆置换,但由于成本和给药便利性,静脉注射免疫球蛋白在病情加重时的使用越来越多。

目的

回顾并严格评估当前关于静脉注射免疫球蛋白和血浆置换对重症肌无力病情恶化患者功能结局影响的证据。

方法

对该目标主题进行了结构化的严格评估。这包括基于临床情景创建结构化问题、全面的文献检索、选择用于综述的证据以及对所选证据进行严格评估。对证据进行了总结并提供了评论。参与者包括顾问和住院神经科医生、医学图书馆员、临床流行病学家以及神经肌肉神经病学领域的内容专家。

结果

选取了一项单盲随机对照试验进行综述,该试验比较了84例重症肌无力病情恶化患者使用静脉注射免疫球蛋白和血浆置换的情况。主要结局指标是治疗后14天的功能状态,通过重症肌无力定量评分进行评估。所有受试者在第14天的重症肌无力定量评分变化为4.0,静脉注射免疫球蛋白组和血浆置换组之间无统计学显著差异。

结论

静脉注射免疫球蛋白和血浆置换在治疗重症肌无力病情恶化方面同样有效。治疗决策可能取决于多个变量,包括是否存在呼吸窘迫、合并症、药物可及性和成本。在真正的重症肌无力危象中,血浆置换可能仍将是首选治疗方法。

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