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一组重症肌无力患者中的抗肌联蛋白抗体

Anti-titin antibodies in a cohort of myasthenia gravis patients.

作者信息

Moura João, Sousa Ana Paula, Samões Raquel, Carneiro Paula, Neves Esmeralda, Silva Ana Martins, Santos Ernestina

机构信息

Department of Neurology, Centro Hospitalar Universitário de Santo António, Porto, Portugal.

Department of Neurophysiology, Centro Hospitalar Universitário de Santo António, Porto, Portugal.

出版信息

J Thorac Dis. 2024 Feb 29;16(2):973-978. doi: 10.21037/jtd-23-94. Epub 2024 Feb 26.

Abstract

BACKGROUND

Anti-titin antibodies have been previously associated with thymoma-associated myasthenia gravis (MG) and a more clinically severe form of MG. While currently only serving as a disease biomarker, its possible utility as an indicator of underlying thymus malignancy may be of value in clinical practice.

METHODS

Data was retrospectively collected and analyzed from 2013 to 2022 using an institutional record of MG patients. Anti-titin antibodies were assessed using Line Blot immunoassay.

RESULTS

From 130 MG cases, 32 (24.6%) were anti-titin positive. Anti-titin positive cases were associated with older age of disease onset [median (IQR): 63.0 (44.3-70.8) 35.5 (24.8-60.8) years] (P<0.01). Thymectomy was performed in 46 (35.4%) MG patients, 12 of which anti-titin positive (26.1%). Thymectomy samples from anti-titin positive patients comprised 10 (83.3%) cases of thymoma and 2 (16.7%) cases of thymus hyperplasia. There was a tendency towards anti-titin positive patients having more thymoma while anti-titin negative displayed more hyperplasia (P<0.01). Anti-titin positivity correlated with thymoma in patients with age of onset bellow 50 years (P=0.028). Anti-titin positivity was significantly associated with generalized MG in the late-onset group (P=0.005).

CONCLUSIONS

The presence of anti-titin antibodies appears to correlate with underlying thymoma in early-onset MG cases and with generalized MG in late-onset cases. Prospective studies are needed to further study this association.

摘要

背景

抗肌联蛋白抗体先前已与胸腺瘤相关的重症肌无力(MG)以及一种临床症状更严重的MG形式相关联。虽然目前仅作为一种疾病生物标志物,但它作为潜在胸腺恶性肿瘤指标的可能用途在临床实践中可能具有价值。

方法

使用MG患者的机构记录,对2013年至2022年的数据进行回顾性收集和分析。使用线性免疫印迹法评估抗肌联蛋白抗体。

结果

在130例MG病例中,32例(24.6%)抗肌联蛋白呈阳性。抗肌联蛋白阳性病例与疾病发病年龄较大相关[中位数(四分位间距):63.0(44.3 - 70.8)岁对35.5(24.8 - 60.8)岁](P<0.01)。46例(35.4%)MG患者接受了胸腺切除术,其中12例抗肌联蛋白呈阳性(26.1%)。抗肌联蛋白阳性患者的胸腺切除样本包括10例(83.3%)胸腺瘤和2例(16.7%)胸腺增生。抗肌联蛋白阳性患者患胸腺瘤的倾向更大,而抗肌联蛋白阴性患者则更多表现为增生(P<0.01)。发病年龄低于50岁的患者中,抗肌联蛋白阳性与胸腺瘤相关(P = 0.028)。在晚发型组中,抗肌联蛋白阳性与全身型MG显著相关(P = 0.005)。

结论

抗肌联蛋白抗体的存在似乎与早发型MG病例中的潜在胸腺瘤以及晚发型病例中的全身型MG相关。需要进行前瞻性研究以进一步探讨这种关联。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1c88/10944723/7715111bdbf6/jtd-16-02-973-f1.jpg

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