中国高龄发病重症肌无力的临床异质性、治疗模式和结局：一项回顾性队列研究

IF 1.8 4区医学 Q3 CLINICAL NEUROLOGY

Journal of Clinical Neuroscience Pub Date : 2025-08-26 DOI:10.1016/j.jocn.2025.111586

Ying Ji , Shuxian Zhou , Xiaoxiao He , Wenbo Li , Xue Zhao , Yingna Zhang , Jing Zhang , Jie Lv , Peipei Liu , Weike Cui , Junhong Yang , Feng Gao

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引用次数: 0

摘要

目的：老年重症肌无力（MG）发病年龄在50岁及以上，通常被称为晚发性MG (LOMG)，其临床特征和预后尚不完全清楚。本研究旨在解决慢性慢性肾病的临床异质性，并阐明极晚发性MG（≥70岁）是否构成一种独特的表型，需要量身定制的治疗方法。方法：我们分析了2009年4月1日至2020年12月31日在MG注册中心登记的2783例患者的数据。MG患者进一步分为两个亚组：50-69岁发病的MG患者（称为早期MG患者）和≥70岁发病的MG患者（称为晚期MG患者，AOMG）。临床特征、诊断测试、治疗和结果在亚组间进行比较。结果982例LOMG患者中，191例（19.4%）为AOMG。MG发病的患者相比,在50 - 69岁,与发病年龄≥70岁(奥美嘉)有较高比例的女性(57.6%比48.7%,p = 0.027),并且更频繁AChR-Ab阳性(81.2%比70.4%,p = 0.001)和Titin-Ab(32.5%比22.5%,p = 0.004),和更有可能与球肌肉参与发病(25.3% vs 14.8%),在疾病进展(53.7% vs 38.6%)。AOMG组重复性神经刺激（RNS）测试阳性率也高于AOMG组（57.1% vs 35.8%, p = 0.018）。胸腺瘤发生率较低（12.1%比29.6%,p = 0.006）， AOMG患者较少接受胸腺切除术（2.6%比31.0%,p < 0.001）。AOMG患者通常需要较少的免疫抑制治疗，并获得相当的功能结果。然而，AOMG组的死亡率明显更高（p = 0.020），主要是由于MG危象。结论与早期lomg相比，saomg表现出明显的临床特征，其特点是累及球的频率更高，胸腺瘤患病率更低，治疗更少，死亡率更高。对于老年MG患者，有针对性的管理策略是必要的。

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Clinical heterogeneity, treatment patterns, and outcomes of advanced-age-onset myasthenia gravis in China: a retrospective cohort study

Objectives

The clinical characteristics and outcomes of myasthenia gravis (MG) with onset at age 50 years or older, which commonly referred to as late-onset MG (LOMG), in elderly patients remain incompletely understood. This study aimed to address the clinical heterogeneity within LOMG and to clarify whether very-late-onset MG (≥70 years) constitutes a distinct phenotype requiring tailored management approaches.

Methods

We analyzed data from 2783 patients enrolled in the MG Registry between April 1, 2009, and December 31, 2020. Patients with LOMG were further stratified into two subgroups: those with MG onset at age 50–69 years (referred to as Early-LOMG), and those with onset at age ≥70 years (referred to as advanced-onset MG, AOMG). Clinical features, diagnostic tests, treatments, and outcomes were compared across subgroups.

Results

Of the 982 patients with LOMG, 191 (19.4 %) were classified as AOMG. Compared to patients with MG onset at age 50–69 years, those with onset at age ≥70 years (AOMG) had a higher proportion of females (57.6 % vs 48.7 %, p = 0.027), and were more frequently positive for AChR-Ab (81.2 % vs 70.4 %, p = 0.001) and Titin-Ab (32.5 % vs 22.5 %, p = 0.004), and were more likely to present with bulbar muscle involvement at onset (25.3 % vs 14.8 %) and during disease progression (53.7 % vs 38.6 %). The positive rate of repetitive nerve stimulation (RNS) testing was also higher in the AOMG group (57.1 % vs 35.8 %, p = 0.018). Thymoma was less frequent (12.1 % vs 29.6 %, p = 0.006), and fewer AOMG patients underwent thymectomy (2.6 % vs 31.0 %, p < 0.001). AOMG patients generally required fewer immunosuppressive therapies and achieved comparable functional outcomes. However, the mortality rate was significantly higher in the AOMG group (p = 0.020), primarily due to MG crisis.

Conclusions

AOMG exhibits distinct clinical features compared to Early-LOMG, characterized by a higher frequency of bulbar involvement, lower thymoma prevalence, less aggressive treatment, and higher mortality. Tailored management strategies are warranted for elderly patients with MG.

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来源期刊

Journal of Clinical Neuroscience 医学-临床神经学

CiteScore

4.50

自引率

0.00%

发文量

402

审稿时长

40 days

期刊介绍： This International journal, Journal of Clinical Neuroscience, publishes articles on clinical neurosurgery and neurology and the related neurosciences such as neuro-pathology, neuro-radiology, neuro-ophthalmology and neuro-physiology. The journal has a broad International perspective, and emphasises the advances occurring in Asia, the Pacific Rim region, Europe and North America. The Journal acts as a focus for publication of major clinical and laboratory research, as well as publishing solicited manuscripts on specific subjects from experts, case reports and other information of interest to clinicians working in the clinical neurosciences.