依夫加替莫德治疗胸腺瘤相关性全身重症肌无力围手术期4例报告

IF 5.9 2区 医学 Q1 IMMUNOLOGY
Frontiers in Immunology Pub Date : 2025-10-02 eCollection Date: 2025-01-01 DOI:10.3389/fimmu.2025.1627584
Liuli Ren, Ling Wei, Song Jiang, Hongru Li, Anguo Chen, Juanjuan Zhang
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引用次数: 0

摘要

背景:胸腺切除术是胸腺瘤相关性重症肌无力的主要治疗方法之一,但胸腺瘤围手术期存在重症肌无力症状急性加重的风险。因此,围手术期的处理非常重要。我们报告了4例胸腺瘤相关的广泛性重症肌无力患者在围手术期前后应用依加替莫德治疗。患者术前肌无力症状得到迅速控制,患者顺利完成手术,术后无肌无力危象及呼吸衰竭。病例报告:诊断为胸腺瘤相关性全身性重症肌无力的4例患者均为乙酰胆碱受体(AChR)抗体阳性,病例2同时为Titin和抗ryr抗体阳性。术前,所有患者均表现出不同程度的肌无力症状,病例2在术前接受糖皮质激素脉冲治疗后出现肌无力危象。4例重症肌无力患者纳入我们的多学科治疗组,在胸腺瘤手术前均接受了1 ~ 2次艾加替莫治疗,重症肌无力症状得到改善,MG-ADL和QMG评分明显降低。4例患者均在全麻下行电视胸腔镜纵隔肿瘤切除术,术后第3 ~ 4次应用依加替莫,围手术期无肌无力危象发生。除病例1术后出现新的肺部感染,肌无力症状出现短暂波动外,其他3例患者均未出现严重并发症。术后1个月和3个月随访显示,3例患者达到最低症状表达(MG-ADL评分≤1)。讨论:依夫加替莫德为胸腺瘤相关性全身重症肌无力患者胸腺瘤围手术期治疗提供了新的见解。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Efgartigimod for patients with thymoma associated generalized myasthenia gravis during the perioperative period: a four-case report.

Efgartigimod for patients with thymoma associated generalized myasthenia gravis during the perioperative period: a four-case report.

Background: Thymectomy is one of the main treatments for thymoma associated myasthenia gravis, but there is a risk of acute exacerbation of myasthenia gravis symptoms during the perioperative period of thymoma. Therefore, perioperative management is very important. We reported four cases of thymoma associated generalized myasthenia gravis who were treated with efgartigimod before and after the perioperative period. The myasthenic symptoms of the patients were quickly controlled before surgery, and the patients successfully completed the surgery without experiencing myasthenic crisis or respiratory failure after surgery.

Case report: Four patients diagnosed with thymoma associated generalized myasthenia gravis were all acetylcholine receptor(AChR) antibody-positive, with case 2 also positive for Titin and anti-RyR antibodies. Prior to surgery, all patients exhibited varying degrees of myasthenic symptoms, with case 2 experiencing a myasthenic crisis induced by glucocorticoid pulse therapy before the operation. Admitted to our multidisciplinary treatment group for myasthenia gravis, all four patients received 1 to 2 treatments with efgartigimod before thymoma surgery, which led to an improvement in myasthenia symptoms and a significant reduction in MG-ADL and QMG scores. All four patients underwent video-assisted thoracoscopic mediastinal tumor resection under general anesthesia, followed by the 3rd to 4th treatments with efgartigimod postoperatively, with no perioperative myasthenic crisis occurring in any of the patients. Apart from case 1, who developed a new pulmonary infection postoperatively and experienced a temporary fluctuation in myasthenia symptoms, the other three patients had no severe complications. Follow-up visits at one month and three months postoperatively showed that three of the patients achieved minimum symptom expression (MG-ADL score ≤1).

Discussion: Efgartigimod provides new insights into the perioperative management of thymomas in patients with thymoma associated generalized myasthenia gravis.

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来源期刊
CiteScore
9.80
自引率
11.00%
发文量
7153
审稿时长
14 weeks
期刊介绍: Frontiers in Immunology is a leading journal in its field, publishing rigorously peer-reviewed research across basic, translational and clinical immunology. This multidisciplinary open-access journal is at the forefront of disseminating and communicating scientific knowledge and impactful discoveries to researchers, academics, clinicians and the public worldwide. Frontiers in Immunology is the official Journal of the International Union of Immunological Societies (IUIS). Encompassing the entire field of Immunology, this journal welcomes papers that investigate basic mechanisms of immune system development and function, with a particular emphasis given to the description of the clinical and immunological phenotype of human immune disorders, and on the definition of their molecular basis.
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