Kwiedor Isabelle, Menacher Martina, Ellßel Monika, Naumann Markus, Bayas Antonios
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.
Ther Adv Neurol Disord. 2024 Dec 23;17:17562864241307687. doi: 10.1177/17562864241307687. eCollection 2024.
In acetylcholine receptor (AChR) antibody-positive generalized myasthenia gravis (gMG), neonatal Fc-receptor (FcRn) inhibition has broadened the therapeutic spectrum. Myasthenic crisis (MC), heralded by an impending myasthenic crisis (iMC), is a critical condition requiring treatments with rapid onset and sustained efficacy. Currently treatments used for iMC, including intravenous immunoglobulins and plasma exchange/immunoadsorption, have limitations, such as delayed onset of action and potential side effects. So far, there is limited data on the use of FcRn inhibitors in the management of impending or manifest MC (mMC). Here, we present a case of AChR antibody-positive gMG with iMC, where subcutaneous administration of the FcRn inhibitor efgartigimod resulted in rapid clinical remission. Within 24 h of administration, the patient exhibited significant improvement in respiratory and bulbar muscle function, preventing progression to manifest MC and the need for mechanical ventilation. This rapid response was accompanied by a marked reduction in AChR antibody level by 89.8% within 4 weeks. This case supports the potential of efgartigimod as a fast-acting and effective treatment option for managing iMC, offering an alternative to existing therapies.
在乙酰胆碱受体（AChR）抗体阳性的全身型重症肌无力（gMG）中，新生儿Fc受体（FcRn）抑制作用拓宽了治疗范围。由即将发生的重症肌无力危象（iMC）预示的重症肌无力危象（MC）是一种危急情况，需要起效迅速且疗效持久的治疗。目前用于iMC的治疗方法，包括静脉注射免疫球蛋白和血浆置换/免疫吸附，存在局限性，如起效延迟和潜在副作用。到目前为止，关于FcRn抑制剂在处理即将发生或已出现的MC（mMC）中的应用数据有限。在此，我们报告一例AChR抗体阳性的gMG合并iMC患者，皮下注射FcRn抑制剂艾加莫德后实现了快速临床缓解。给药后24小时内，患者呼吸和延髓肌肉功能显著改善，避免进展为显性MC以及避免了机械通气的需求。这种快速反应伴随着4周内AChR抗体水平显著降低89.8%。该病例支持了艾加莫德作为一种快速起效且有效的治疗iMC的选择的潜力，为现有疗法提供了替代方案。
