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胸腺瘤中的亚临床型重症肌无力

Subclinical myasthenia gravis in thymomas.

作者信息

Marcuse Florit, Hochstenbag Monique, Hoeijmakers Janneke G J, Hamid Myrurgia Abdul, Damoiseaux Jan, Maessen Jos, De Baets Marc

机构信息

Department of Pulmonology, Maastricht University Medical Center+, Maastricht, the Netherlands; School for Mental Health and Neuroscience, Maastricht University, Maastricht, the Netherlands.

Department of Pulmonology, Maastricht University Medical Center+, Maastricht, the Netherlands.

出版信息

Lung Cancer. 2021 Feb;152:143-148. doi: 10.1016/j.lungcan.2020.12.010. Epub 2020 Dec 17.

DOI:10.1016/j.lungcan.2020.12.010
PMID:33401082
Abstract

BACKGROUND

A proportion of thymoma-patients without a history of myasthenia gravis (MG) before thymectomy, appears to have positive anti-AChR-antibodies in the serum. These subclinical MG-patients could be underdiagnosed because analyzation of anti-AChR-antibodies in thymomas is not always performed in patients who did not experience neurological symptoms. The prevalence and long-term outcomes of subclinical MG are never described in literature yet.

METHODS

We retrospectively analyzed 398 consecutive patients who underwent a robotic-assisted thoracoscopic surgery at the Maastricht University Medical Center+ (MUMC+) between April 2004 and December 2018. In the MUMC+, a robotic approach is the standard surgical approach in patients with thymic diseases. Inclusion criteria were thymomas, thymectomy performed in the MUMC + with a follow-up of at least one year and age above 18 years old. Exclusion criteria were patients with thymic carcinomas, refused participation, or those who were lost to follow-up.

RESULTS

Of the 102 included thymoma-patients, 87 patients (85 %) were tested for anti-AChR-antibodies before thymectomy, of which 57 patients were diagnosed with clinical MG and seven subclinical MG-patients were found. Of the 15 patients who were not tested for anti-AChR-antibodies, four more subclinical MG-patients were discovered in the years after thymectomy. The median follow-up time was 62 months. In total, 11 subclinical MG-patients were found, with a mean age of 54 years and predominantly females (64 %). Ten subclinical MG-patients (91 %) developed clinical-MG, within six years after thymectomy. Immunosuppressive drugs were prescribed in five patients. Four patients were diagnosed with a recurrence of the thymoma. No surgical mortality was reported. Two patients died due to a myasthenic crisis.

CONCLUSIONS

The prevalence of subclinical MG in thymomas was found to be 10.8 %. One in four patients who experienced no neurological symptoms before thymectomy, appeared to have anti-AChR-antibodies and 91 % of these patients developed clinical MG within six years after the thymectomy. Analyzing anti-AChR-antibodies in the serum is recommended in all suspected thymomas before a thymectomy is performed.

摘要

背景

一部分在胸腺切除术前无重症肌无力(MG)病史的胸腺瘤患者,血清中抗乙酰胆碱受体(AChR)抗体呈阳性。这些亚临床MG患者可能未被充分诊断,因为在未出现神经症状的患者中,并非总是对胸腺瘤中的抗AChR抗体进行分析。亚临床MG的患病率和长期预后在文献中尚未有描述。

方法

我们回顾性分析了2004年4月至2018年12月在马斯特里赫特大学医学中心+(MUMC+)接受机器人辅助胸腔镜手术的398例连续患者。在MUMC+,机器人手术方法是胸腺疾病患者的标准手术方法。纳入标准为胸腺瘤、在MUMC+进行胸腺切除术且随访至少一年以及年龄在18岁以上。排除标准为胸腺癌患者、拒绝参与或失访者。

结果

在纳入的102例胸腺瘤患者中,87例(85%)在胸腺切除术前检测了抗AChR抗体,其中57例被诊断为临床MG,发现7例亚临床MG患者。在未检测抗AChR抗体的15例患者中,胸腺切除术后数年又发现4例亚临床MG患者。中位随访时间为62个月。总共发现11例亚临床MG患者,平均年龄54岁,以女性为主(64%)。10例亚临床MG患者(91%)在胸腺切除术后6年内发展为临床MG。5例患者使用了免疫抑制药物。4例患者被诊断为胸腺瘤复发。未报告手术死亡病例。2例患者死于重症肌无力危象。

结论

发现胸腺瘤中亚临床MG的患病率为10.8%。四分之一在胸腺切除术前无神经症状的患者似乎有抗AChR抗体,其中91%的患者在胸腺切除术后6年内发展为临床MG。建议在所有疑似胸腺瘤患者进行胸腺切除术前检测血清中的抗AChR抗体。

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