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大剂量静脉注射免疫球蛋白治疗重症肌无力

High-dose intravenous immunoglobulin therapy for myasthenia gravis.

作者信息

Jongen J L, van Doorn P A, van der Meché F G

机构信息

Department of Neurology, University Hospital Rotterdam-Dijkzigt, The Netherlands.

出版信息

J Neurol. 1998 Jan;245(1):26-31. doi: 10.1007/s004150050170.

Abstract

The objective of this open, retrospective study was to investigate whether intravenous immunoglobulin (IVIg) could induce a clinically obvious improvement in patients with generalized myasthenia gravis (MG), as judged by MG functional status. Fourteen patients with generalized MG were treated during at least one episode with 0.4 g IVIg per kilogram body weight per day for 5 consecutive days. Patients with confounding variables were excluded; this left 11 patients (16 episodes) to be further analysed. We defined improvement as at least a one-step improvement in MG functional status (according to the University of Virginia's Modification of Osserman's classification). Of the treatment episodes, 56% were classified as positive responses. If improvement occurred, onset of improvement started after 3 (1-12) days and peak effect was reached after 7 (4-30) days (median and range). All four patients who required artificial ventilation could be weaned from it 8.5 (6-11) days after the start of IVIg (median and range). Of the patients treated on two occasions, only one patient had a positive response during both. In MG functional status 5, improvement was observed during five of seven episodes. None of the patients with MG functional status 3 responded. Patients with an acute relapse of MG seemed to respond equally well to IVIg compared with patients with subacute deterioration/ chronic-static state (50% versus 60%). The MG functional status at the start of IVIg and on the day of maximal improvement was compared for all episodes together, and significant improvement was noted (P = 0.0052). We did not see any serious side-effects after IVIg treatment. This retrospective analysis suggests that high-dose IVIg is an effective therapy in some patients with deterioration of generalized MG. If improvement occurs, it starts within a few days of the onset of IVIg and the effect seems to peak within 2 weeks.

摘要

这项开放性回顾性研究的目的是,根据重症肌无力(MG)功能状态,调查静脉注射免疫球蛋白(IVIg)能否使全身型重症肌无力患者在临床上有明显改善。14例全身型MG患者在至少1次发作期间接受了每天每千克体重0.4 g IVIg的治疗,连续5天。排除有混杂变量的患者;剩下11例患者(16次发作)进行进一步分析。我们将改善定义为MG功能状态至少提高一个等级(根据弗吉尼亚大学对奥斯默曼分类法的修订)。在治疗发作中,56%被归类为阳性反应。如果出现改善,改善在3(1 - 12)天开始,7(4 - 30)天达到峰值效应(中位数和范围)。所有4例需要人工通气的患者在IVIg开始后8.5(6 - 11)天可以撤机(中位数和范围)。在接受两次治疗的患者中,只有1例患者两次治疗均有阳性反应。在MG功能状态为5级的患者中,7次发作中有5次观察到改善。MG功能状态为3级的患者均无反应。MG急性复发的患者与亚急性恶化/慢性静止状态的患者对IVIg的反应似乎同样良好(50%对60%)。将所有发作的IVIg开始时和最大改善日的MG功能状态进行比较,发现有显著改善(P = 0.0052)。IVIg治疗后我们未观察到任何严重副作用。这项回顾性分析表明,高剂量IVIg对一些全身型MG病情恶化的患者是一种有效的治疗方法。如果出现改善,在IVIg开始后几天内开始,效果似乎在2周内达到峰值。

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