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在血清学阳性的全身型重症肌无力中,补体抑制作为对即将发生的肌无力危象的挽救治疗以及对频繁复发的即将发生的肌无力危象的有效治疗——病例系列

Complement inhibition in seropositive generalized myasthenia gravis as rescue therapy in impending and effective treatment in frequently recurring impending myasthenic crisis-a case series.

作者信息

Menacher Martina, Ellssel Monika, Kwiedor Isabelle, Naumann Markus, Bayas Antonios

机构信息

Department of Neurology, Faculty of Medicine, University of Augsburg, Augsburg, Germany.

Department of Neurology, Faculty of Medicine, University of Augsburg, Stenglinstrasse 2, Augsburg 86156, Germany.

出版信息

Ther Adv Neurol Disord. 2024 Dec 23;17:17562864241301361. doi: 10.1177/17562864241301361. eCollection 2024.

Abstract

In seropositive myasthenia gravis (MG), complement inhibition has been shown to be an effective and a fast-acting therapeutic option. Myasthenic crisis (MC), usually preceded by impending MC, is a life-threatening complication requiring highly effective treatments with rapid onset of action. Currently used treatment options of MC are limited, consisting mainly of symptomatic and immune therapies, that is, intravenous immunoglobulins and plasma exchange/immunoadsorption. So far, there is only very limited data on complement inhibitors in impending or manifest MC or termination of frequently recurring impending crises. Here, we report three cases of acetylcholine receptor antibody positive MG, two with impending and one case suffering from high-frequency impending MC, where complement inhibition with eculizumab or ravulizumab resulted in a rapid and sustained remission. Meningococcal vaccination, mandatory when using complement inhibitors, did not result in symptom-worsening or manifest MC.

摘要

在血清反应阳性的重症肌无力(MG)中,补体抑制已被证明是一种有效且起效迅速的治疗选择。肌无力危象(MC)通常在即将发生MC之前出现,是一种危及生命的并发症,需要起效迅速的高效治疗。目前用于治疗MC的选择有限,主要包括对症治疗和免疫治疗,即静脉注射免疫球蛋白和血浆置换/免疫吸附。到目前为止,关于补体抑制剂在即将发生或明显的MC中或终止频繁复发的即将发生的危象方面的数据非常有限。在此,我们报告3例乙酰胆碱受体抗体阳性的MG患者,其中2例即将发生危象,1例患有高频即将发生的MC,使用依库珠单抗或ravulizumab进行补体抑制导致快速且持续的缓解。使用补体抑制剂时必须进行的脑膜炎球菌疫苗接种并未导致症状恶化或明显的MC。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/944e/11672472/e97a1bc9bd74/10.1177_17562864241301361-fig1.jpg

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