Takenobu Yohei, Ikeda Kazutoshi, Hasebe Sachiko, Nomura Noriko, Tamaki Shunsuke, Yukawa Kayoko, Miyahara Junichi, Yamakawa Kentaro, Inoue Manabu
Department of Neurology, Osaka Red Cross Hospital, Osaka, Japan.
Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto, Japan.
Immunol Med. 2025 May 6:1-6. doi: 10.1080/25785826.2025.2500698.
Myasthenic crisis (MC) represents the most severe and life-threatening complication of myasthenia gravis (MG). Some patients exhibit refractory responses to conventional immunotherapies, including intravenous immunoglobulin and plasma exchange. This report describes a patient with MC refractory to repetitive high-dose steroids and intravenous immunoglobulin, requiring ventilator support. Within 2 days of eculizumab administration, significant improvement enabled ventilator discontinuation. Subsequent doses further ameliorated limb and pharyngeal weakness, leading to independence. A literature review that identified ten cases reported across five publications highlighted the favorable outcomes achieved with eculizumab in refractory MC, while concomitant respiratory infection was shown to complicate the recovery from MG-related respiratory failure. Although the randomized controlled trials have excluded MC cases, eculizumab has emerged as a promising option for rescue therapy in refractory MC. Larger studies that specifically include MC cases are warranted.
重症肌无力危象(MC)是重症肌无力(MG)最严重且危及生命的并发症。一些患者对包括静脉注射免疫球蛋白和血浆置换在内的传统免疫疗法表现出难治性反应。本报告描述了一名对重复高剂量类固醇和静脉注射免疫球蛋白难治的MC患者,需要呼吸机支持。在使用依库珠单抗2天内,病情显著改善,得以停用呼吸机。后续剂量进一步改善了肢体和咽部无力,使其能够独立。一项文献综述在五篇出版物中确定了十例报告病例,突出了依库珠单抗在难治性MC中取得的良好结果,同时显示合并呼吸道感染会使MG相关呼吸衰竭的恢复复杂化。尽管随机对照试验排除了MC病例,但依库珠单抗已成为难治性MC抢救治疗的一个有前景的选择。有必要开展专门纳入MC病例的更大规模研究。