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重症肌无力中抗乙酰胆碱受体抗体与临床亚型及疾病严重程度的关联

Association of blocking acetylcholine receptor antibodies with clinical subtypes and disease severity in myasthenia gravis.

作者信息

Liu Kuan, Yang Wenbo, Liu Xiaoni, Yu Hai, Zhu Dongqin, Li Yarong, Chen Xiangjun, Zhang Xiang

机构信息

Department of Neurology, Huashan Hospital, Fudan University and Institute of Neurology, Fudan University, National Center for Neurological Disorders, Shanghai, China.

Department of Neurology, Huashan Hospital, Fudan University and Institute of Neurology, Fudan University, National Center for Neurological Disorders, Shanghai, China.

出版信息

Clin Chim Acta. 2025 Sep 1;577:120449. doi: 10.1016/j.cca.2025.120449. Epub 2025 Jun 25.

Abstract

BACKGROUND AND AIM

The identification of serum antibodies, particularly acetylcholine receptor antibodies (AChR-ab), is essential for diagnosing myasthenia gravis (MG). However, the clinical significance of blocking AChR-abs (bAChR-ab) and their correlation with disease severity remain underexplored. This study aims to evaluate the distribution of bAChR-ab across MG subtypes and their correlation with clinical severity.

METHODS

148 MG patients were recruited from Huashan Hospital, Fudan University. The positive ratio of nAChR-ab and bAChR-ab was evaluated, and the correlations of bAChR-ab inhibition rate were analyzed in relation to clinical severity.

RESULTS

Of total148 patients diagnosed with MG, 76 (51.35%) classified as ocular myasthenia gravis (OMG) and 72 (48.65%) as generalized myasthenia gravis (GMG). Thymic abnormalities were more common in GMG, with 19.44% of GMG patients having thymoma compared to 9.21% of OMG patients, though the difference was not statistically significant. 43.24% of patients tested positive for bAChR-ab, with a significantly higher positivity rate observed in GMG (58.33%) compared to patients with OMG. Elevated bAChR-ab inhibition rate significantly correlated with disease severity, while nAChR-ab did not demonstrate any correlation with either the MGC score or the ADL score in either groups.

CONCLUSION

bAChR-ab was exclusively detected and showed a strong association with severe clinical manifestations, including myasthenic crises. These findings highlight bAChR-ab as a potential biomarker for monitoring MG severity. The role of bAchR-ab in seronegative MG will be further verified. Larger, multi-center studies warrant to validate these results and refine diagnostic and therapeutic strategies for MG management.

摘要

背景与目的

血清抗体的鉴定,尤其是乙酰胆碱受体抗体(AChR-ab),对于重症肌无力(MG)的诊断至关重要。然而,阻断型AChR抗体(bAChR-ab)的临床意义及其与疾病严重程度的相关性仍未得到充分研究。本研究旨在评估bAChR-ab在MG各亚型中的分布及其与临床严重程度的相关性。

方法

从复旦大学附属华山医院招募了148例MG患者。评估了nAChR-ab和bAChR-ab的阳性率,并分析了bAChR-ab抑制率与临床严重程度的相关性。

结果

在总共148例诊断为MG的患者中,76例(51.35%)归类为眼肌型重症肌无力(OMG),72例(48.65%)归类为全身型重症肌无力(GMG)。胸腺异常在GMG中更为常见,19.44%的GMG患者患有胸腺瘤,而OMG患者中这一比例为9.21%,尽管差异无统计学意义。43.24%的患者bAChR-ab检测呈阳性,GMG患者的阳性率(58.33%)显著高于OMG患者。bAChR-ab抑制率升高与疾病严重程度显著相关,而nAChR-ab在两组中与MGC评分或ADL评分均无相关性。

结论

仅检测到bAChR-ab,且其与包括肌无力危象在内的严重临床表现密切相关。这些发现突出了bAChR-ab作为监测MG严重程度的潜在生物标志物的作用。bAchR-ab在血清阴性MG中的作用将进一步得到验证。需要更大规模的多中心研究来验证这些结果,并完善MG管理的诊断和治疗策略。

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