Yan Jinyi, Choi Kalam, Fu Peicai, Li Yue, Li Zhijun
Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, Hubei, China.
BMC Neurol. 2025 Sep 25;25(1):384. doi: 10.1186/s12883-025-04438-4.
This case demonstrates the efficacy and safety of efgartigimod in managing an acute exacerbation of myasthenia gravis (MG) with comorbid Castleman disease, particularly during the perioperative care of retroperitoneal mass resection.
A 23-year-old female was admitted with the discovery of a retroperitoneal Mass, confirmed via abdominal enhanced CT and MRI. The patient had generalized MG since 2017, with controlled but fluctuating symptoms.
Preoperative assessment showed albumin levels were slightly reduced and respiratory function demonstrated severe restrictive ventilation impairment. There was acute exacerbation of muscle weakness (MG Foundation of America (MGFA) class IVb, MG-activities of daily living (ADL) score 8, and quantitative MG (QMG) score 20) with respiratory and medullary symptoms. Treatment with efgartigimod (10 mg/kg/week), prednisone 15 mg/day and pyridostigmine 270 mg/day was initiated. The symptoms improved substantially after four infusions (MG-ADL score 0, QMG score 6), and the lung function recovered. The patient continued efgartigimod treatment and underwent retroperitoneal mass resection. The mass showed massive lymph node hyperplasia (Castleman's disease, mixed type). Postoperatively, the patient experienced no infection, MG exacerbation, or other complications.
Efgartigimod had a rapid onset of action, swiftly improved symptoms during acute MG exacerbation, and maintained symptom stability during the perioperative care.
本病例展示了艾加莫德在治疗合并Castleman病的重症肌无力（MG）急性加重时的有效性和安全性，特别是在腹膜后肿块切除的围手术期护理期间。
一名23岁女性因发现腹膜后肿块入院，经腹部增强CT和MRI确诊。该患者自2017年起患有全身性MG，症状得到控制但有波动。
术前评估显示白蛋白水平略有降低，呼吸功能表现为严重的限制性通气障碍。出现肌无力急性加重（美国MG基金会（MGFA）IVb级，MG日常生活活动（ADL）评分8分，定量MG（QMG）评分20分），伴有呼吸和延髓症状。开始使用艾加莫德（10mg/kg/周）、泼尼松15mg/天和吡啶斯的明270mg/天进行治疗。四次输注后症状显著改善（MG-ADL评分0分，QMG评分6分），肺功能恢复。患者继续接受艾加莫德治疗并接受了腹膜后肿块切除术。肿块显示为巨大淋巴结增生（Castleman病，混合型）。术后，患者未发生感染、MG加重或其他并发症。
艾加莫德起效迅速，在MG急性加重期间迅速改善症状，并在围手术期护理期间维持症状稳定。
