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中国晚发型重症肌无力的临床特征与预后

Clinical characteristics and prognosis of very late-onset myasthenia gravis in China.

作者信息

Tang Yong-Lan, Ruan Zhe, Su Yue, Guo Rong-Jing, Gao Ting, Liu Yu, Li Huan-Huan, Sun Chao, Li Zhu-Yi, Chang Ting

机构信息

Department of Neurology, Tangdu Hospital, The Fourth Military Medical University, Xi'an 710038, China.

Department of Neurology, Tangdu Hospital, The Fourth Military Medical University, Xi'an 710038, China.

出版信息

Neuromuscul Disord. 2023 Apr;33(4):358-366. doi: 10.1016/j.nmd.2023.02.013. Epub 2023 Mar 4.

Abstract

Alteration in onset-age distribution in myasthenia gravis (MG) and its increasing prevalence among the elderly underscores the need for a better understanding of the clinical course of MG and the establishment of personalized treatment. In this study we reviewed the demographics, clinical profile, and treatment of MG. Based on onset age, eligible patients were classified as early-onset MG (onset age ≥18 and <50 years), late-onset MG (onset age ≥50 and <65 years), and very late-onset MG (onset age ≥65 years). Overall, 1160 eligible patients were enrolled. Patients with late- and very late-onset MG showed a male predominance (P=0.02), ocular MG subtype (P=0.001), and seropositivity for acetylcholine receptors and titin antibodies (P<0.001). In very late-onset MG, a lower proportion of patients retained minimal manifestations status or better, a higher proportion of patients had MG-related deaths (P<0.001), and a shorter maintenance time of minimal manifestation status or better was seen at the last follow-up (P=0.007) than that in patients with early- and late-onset MG. Non-immunotherapy may associated with a poor prognosis in patients in the very late-onset group. Further studies on very late-onset MG patients should be performed to evaluate the relationship between immunotherapy and prognosis.

摘要

重症肌无力(MG)发病年龄分布的改变及其在老年人中患病率的增加,凸显了更好地了解MG临床病程并建立个性化治疗的必要性。在本研究中,我们回顾了MG患者的人口统计学、临床特征和治疗情况。根据发病年龄,符合条件的患者被分为早发型MG(发病年龄≥18岁且<50岁)、晚发型MG(发病年龄≥50岁且<65岁)和极晚发型MG(发病年龄≥65岁)。总体而言,共纳入了1160例符合条件的患者。晚发型和极晚发型MG患者表现出男性占优势(P=0.02)、眼肌型MG亚型(P=0.001)以及乙酰胆碱受体和肌联蛋白抗体血清阳性(P<0.001)。在极晚发型MG中,与早发型和晚发型MG患者相比,维持最小表现状态或更好状态的患者比例更低,MG相关死亡的患者比例更高(P<0.001),并且在最后一次随访时维持最小表现状态或更好状态的时间更短(P=0.007)。非免疫治疗可能与极晚发型组患者的预后不良相关。应对极晚发型MG患者进行进一步研究,以评估免疫治疗与预后之间的关系。

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