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胸腺切除术后重症肌无力患者诊断延迟及临床病程的性别差异

Differences Between Females and Males in the Diagnostic Delay and Clinical Course of Thymectomised Myasthenia Gravis.

作者信息

Myllynen Chris, Tuulasvaara Anni, Atula Sari, Laakso Sini M

机构信息

Department of Neurosciences, University of Helsinki, Helsinki, Finland.

Department of Neurology, Brain Center, Helsinki University Hospital, Helsinki, Finland.

出版信息

Eur J Neurol. 2025 May;32(5):e70114. doi: 10.1111/ene.70114.

Abstract

INTRODUCTION

Myasthenia gravis (MG) is an autoimmune disease presenting typically at an earlier age in females compared to males. However, whether sex affects the diagnostic delay and clinical course of MG has not been extensively explored in systematic cohorts.

METHODS

We conducted a retrospective single-center cohort study on 251 thymectomized MG patients, including 124 males and 127 females. Mean follow-up was 10.7 (±9.1) years post-thymectomy. We analyzed factors associated with longer diagnostic delay and the type of first-onset symptoms (ocular vs. generalized). For 195 patients with nonthymomatous generalized MG (gMG) pre-thymectomy, we estimated the effect of diagnostic delay, symptoms at onset, and sex on reaching complete stable remission (CSR) or minimal need for medication (MNM) during postoperative follow-up. We also assessed their effect on the overall need for in-hospital treatments, immunosuppressant use, and pyridostigmine dose at the last follow-up visit.

RESULTS

Generalized symptoms at onset were more frequent for females than for males (n = 90, 70.9% vs. n = 65, 52.4%, respectively; p < 0.001). Diagnostic delay was significantly longer in females (6.0 months [0-117.5] vs. 3.2 months in males [0.1-84.0]; p = 0.012). First-onset symptoms and diagnostic delay did not affect the post-thymectomy prognosis of gMG. However, females achieved CSR (17.1% vs. 4.5%; p = 0.006) and MNM (30.8% vs. 16.9%; p = 0.029) more frequently and required fewer in-hospital treatments (40.6% vs. 55.2%; p = 0.010) and immunosuppressants (29.2% vs. 61.7%; p < 0.001) than males.

CONCLUSIONS

We report sex-related differences in symptoms at MG onset, length of diagnostic delay, and prognosis, the origins of which should be further studied.

摘要

引言

重症肌无力(MG)是一种自身免疫性疾病,女性发病年龄通常比男性更早。然而,性别是否会影响MG的诊断延迟和临床病程,尚未在系统性队列研究中得到广泛探讨。

方法

我们对251例接受胸腺切除术的MG患者进行了一项回顾性单中心队列研究,其中包括124例男性和127例女性。胸腺切除术后的平均随访时间为10.7(±9.1)年。我们分析了与较长诊断延迟相关的因素以及首发症状的类型(眼肌型与全身型)。对于195例胸腺切除术前患有非胸腺瘤性全身型MG(gMG)的患者,我们评估了诊断延迟、起病时的症状和性别对术后随访期间达到完全稳定缓解(CSR)或药物需求最小化(MNM)的影响。我们还评估了它们对最后一次随访时住院治疗总需求、免疫抑制剂使用情况和吡啶斯的明剂量的影响。

结果

女性起病时出现全身症状的频率高于男性(分别为n = 90,70.9% 与n = 65,52.4%;p < 0.001)。女性的诊断延迟明显更长(6.0个月[0 - 117.5],而男性为3.2个月[0.1 - 84.0];p = 0.012)。首发症状和诊断延迟并不影响gMG患者胸腺切除术后的预后。然而,女性比男性更频繁地实现了CSR(17.1% 对4.5%;p = 0.006)和MNM(30.8% 对16.9%;p = 0.029),并且住院治疗需求更少(40.6% 对55.2%;p = 0.010),免疫抑制剂使用更少(29.2% 对61.7%;p < 0.001)。

结论

我们报告了MG起病时的症状、诊断延迟长度和预后方面存在性别差异,其根源有待进一步研究。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2a23/12051382/ea9032faa5df/ENE-32-e70114-g001.jpg

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