Perioperative Efgartigimod treatment for acute myasthenia gravis exacerbation with retroperitoneal mass: a case report.

IF 2.2 3区 医学 Q3 CLINICAL NEUROLOGY
Jinyi Yan, Kalam Choi, Peicai Fu, Yue Li, Zhijun Li
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引用次数: 0

Abstract

Objective: 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.

Methods: 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.

Results: 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.

Discussion: Efgartigimod had a rapid onset of action, swiftly improved symptoms during acute MG exacerbation, and maintained symptom stability during the perioperative care.

依加替莫德围手术期治疗急性重症肌无力加重伴腹膜后肿块1例。
目的:本病例证明了依加替莫德治疗合并Castleman病的重症肌无力急性加重的有效性和安全性,特别是在腹膜后肿块切除术的围手术期护理中。方法:一名23岁女性因发现腹膜后肿块而入院,经腹部增强CT和MRI证实。患者自2017年以来患有全身性MG,症状可控但波动。结果:术前评估显示白蛋白水平轻微下降,呼吸功能表现出严重的限制性通气障碍。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)伴有呼吸和髓系症状。开始使用埃加替莫德(10 mg/kg/周)、强的松15 mg/天和吡哆斯的明270 mg/天治疗。4次输注后症状明显改善(MG-ADL评分0分,QMG评分6分),肺功能恢复。患者继续依加替莫治疗并行腹膜后肿物切除术。肿块表现为大量淋巴结增生(Castleman病,混合型)。术后,患者未出现感染、MG加重或其他并发症。讨论:依加替莫德起效快,急性MG加重时症状迅速改善,围手术期护理中症状保持稳定。
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来源期刊
BMC Neurology
BMC Neurology 医学-临床神经学
CiteScore
4.20
自引率
0.00%
发文量
428
审稿时长
3-8 weeks
期刊介绍: BMC Neurology is an open access, peer-reviewed journal that considers articles on all aspects of the prevention, diagnosis and management of neurological disorders, as well as related molecular genetics, pathophysiology, and epidemiology.
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