补体抑制对血清阳性全身性重症肌无力危象的抢救和有效治疗——病例系列。

IF 4.7 2区 医学 Q1 CLINICAL NEUROLOGY Therapeutic Advances in Neurological Disorders Pub Date : 2024-12-23 eCollection Date: 2024-01-01 DOI:10.1177/17562864241301361
Martina Menacher, Monika Ellssel, Isabelle Kwiedor, Markus Naumann, Antonios Bayas
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引用次数: 0

摘要

在血清学阳性重症肌无力(MG)中,补体抑制已被证明是一种有效和快速的治疗选择。肌无力危象(MC)是一种危及生命的并发症,需要快速有效的治疗。目前使用的治疗方案有限,主要是对症治疗和免疫治疗,即静脉注射免疫球蛋白和血浆交换/免疫吸附。到目前为止,只有非常有限的补体抑制剂在即将发生或明显的MC或频繁复发的即将发生的危机终止的数据。在这里,我们报告了3例乙酰胆碱受体抗体阳性MG, 2例与迫在眉睫和1例患有高频迫在眉睫的MC,其中补体抑制eculizumab或ravulizumab导致快速和持续的缓解。当使用补体抑制剂时,必须接种脑膜炎球菌疫苗,未导致症状恶化或明显的MC。
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Complement inhibition in seropositive generalized myasthenia gravis as rescue therapy in impending and effective treatment in frequently recurring impending myasthenic crisis-a case series.

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.

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来源期刊
CiteScore
8.30
自引率
1.70%
发文量
62
审稿时长
15 weeks
期刊介绍: Therapeutic Advances in Neurological Disorders is a peer-reviewed, open access journal delivering the highest quality articles, reviews, and scholarly comment on pioneering efforts and innovative studies across all areas of neurology. The journal has a strong clinical and pharmacological focus and is aimed at clinicians and researchers in neurology, providing a forum in print and online for publishing the highest quality articles in this area.
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