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重症肌无力患者的临床特征与预后

Clinical features and outcomes of patients with myasthenia gravis.

作者信息

Alanazy Mohammed H

机构信息

Division of Neurology, Department of Internal Medicine, King Saud University, Riyadh, Kingdom of Saudi Arabia. E-mail:

出版信息

Neurosciences (Riyadh). 2019 Jul;24(3):176-184. doi: 10.17712/nsj.2019.3.20190011.

Abstract

OBJECTIVE

To report clinical and laboratory features and outcomes of patients with autoimmune myasthenia gravis (MG) recruited from a single center in Saudi Arabia.

METHODS

We retrospectively reviewed prospectively collected data obtained from MG patients who have undergone examination and follow-up at our neuromuscular clinic between August 1, 2014 and January 31, 2019.

RESULTS

Ninety-five patients (55 females) were included. The mean age of onset of MG was 40.5+/-18.1 years in males and 31.3+/-15 years in females (p=0.009). The mean duration of follow-up at our clinic was 34.7+/-14.1 months, while the mean duration since MG onset was 8.0+/-7.2 years. Of all patients, 92.6% had generalized MG, 82.1% had acetylcholine receptor (AChR) antibodies, 4.2% had muscle-specific tyrosine kinase (MuSK) antibodies, 78.9% had early-onset MG with no second peak after age of 50 years, 22.1% had myasthenia crisis, 12.6% had refractory MG, 31.6% had thymic hyperplasia, 10.5% had thymoma, and 61.1% required more than or equal 2 immunosuppressive therapies. At the last follow-up, 93 patients had achieved an optimal outcome (MG Foundation of America classification less than or equal II). No patient with double-seronegative (dSN)-MG had thymoma, needed rituximab or intravenous immunoglobulin maintenance therapy, or was classified as refractory MG.

CONCLUSION

Contrary to other studies, we did not observe a second-peak of MG onset. Clinical outcomes were favorable in the majority of our patients.

摘要

目的

报告从沙特阿拉伯单一中心招募的自身免疫性重症肌无力(MG)患者的临床、实验室特征及预后。

方法

我们回顾性分析了前瞻性收集的数据,这些数据来自于2014年8月1日至2019年1月31日期间在我们神经肌肉诊所接受检查和随访的MG患者。

结果

共纳入95例患者(55例女性)。男性MG发病的平均年龄为40.5±18.1岁,女性为31.3±15岁(p=0.009)。在我们诊所的平均随访时间为34.7±14.1个月,而自MG发病以来的平均时间为8.0±7.2年。所有患者中,92.6%为全身型MG,82.1%有乙酰胆碱受体(AChR)抗体,4.2%有肌肉特异性酪氨酸激酶(MuSK)抗体,78.9%为早发型MG且50岁后无第二个发病高峰,22.1%有重症肌无力危象,12.6%为难治性MG,31.6%有胸腺增生,10.5%有胸腺瘤,61.1%需要两种及以上免疫抑制治疗。在最后一次随访时,93例患者达到了最佳预后(美国重症肌无力基金会分级小于或等于II级)。双血清阴性(dSN)-MG患者中无一人有胸腺瘤,需要利妥昔单抗或静脉注射免疫球蛋白维持治疗,或被归类为难治性MG。

结论

与其他研究相反,我们未观察到MG发病的第二个高峰。我们大多数患者的临床预后良好。

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