AChR-Ab阳性极晚发型重症肌无力患者的短期和长期预后

IF 4.7 2区 医学 Q1 CLINICAL NEUROLOGY Therapeutic Advances in Neurological Disorders Pub Date : 2025-01-08 eCollection Date: 2025-01-01 DOI:10.1177/17562864241309793
Nairong Xie, Qing Liu, Qi Wen, Yaye Wang, Haoran Liu, Yuting Jiang, Yan Lu, Li Di, Min Wang, Wenjia Zhu, Xinmei Wen, Xuxiang Zhang, Xin-Ming Shen, Yuwei Da
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引用次数: 0

摘要

背景:极晚发性重症肌无力(vomg)是指65岁及以上发病的重症肌无力(MG)。目前对VLOMG预后的研究仍然有限,特别是对影响预后的因素的研究。目的:确定影响发病年龄大于或等于65岁的MG患者短期和长期预后的临床因素。设计:这是AChR-ab阳性vomg患者的单中心,回顾性队列研究,根据发病年龄分为两个亚组:亚极晚发病MG (s - vomg,发病年龄大于或大于65岁)和方法:总共包括93名患者,包括75名在s - vomg组和18名在SLOMG组。回顾临床、治疗和预后数据,并使用Cox回归模型确定影响短期和长期预后的因素。结果:两组患者的特征基本平衡。总体而言,49.5%的患者在6个月内达到最小症状表达(MSE), 86%在24个月内达到最小症状表达。在6个月(p = 0.635)和24个月(p = 0.714)时,两组间达到MSE的比例无显著差异。S-VLOMG组和SLOMG组达到MSE的中位时间也具有可比性(199.0天vs 280.5天,p = 0.463)。低基线MG-ADL评分和类固醇治疗与较好的短期预后相关(分别为p = 0.007和p = 0.002)。对于长期预后,基线球和肢体受损伤、开始治疗时间、使用免疫抑制剂是显著因素(p = 0.025, p = 0.004, p = 0.025, p)。结论:本研究表明,AChR-ab阳性vomg患者预后良好,对药物反应良好,年龄和合共病无显著影响。
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Short-term and long-term prognoses in AChR-Ab positive very-late-onset myasthenia gravis patients.

Background: Very-late-onset myasthenia gravis (VLOMG) refers to myasthenia gravis (MG) with onset at age 65 or older. Current research on VLOMG prognosis remains limited, especially regarding factors influencing outcomes.

Objectives: To identify the clinical factors that affect the short- and long-term prognosis of MG patients with an onset age ⩾65 years.

Design: This was a single-center, retrospective cohort study of AChR-ab positive VLOMG patients, classified into two subgroups based on age of onset: sub-very-late-onset MG (S-VLOMG, onset age ⩾65 and <75 years), and super-late-onset MG (SLOMG, onset age ⩾75 years).

Methods: A total of 93 patients were included, including 75 in the S-VLOMG group and 18 in the SLOMG group. Clinical, therapeutic, and prognosis data were reviewed, and the Cox regression model was used to identify factors influencing short- and long-term prognosis.

Results: Patient characteristics were well balanced between the groups. Overall, 49.5% of patients achieved minimal symptom expression (MSE) within 6 months and 86% within 24 months. There was no significant difference between the groups in the proportion achieving MSE at 6 months (p = 0.635) or 24 months (p = 0.714). The median time to achieve MSE was also comparable between the S-VLOMG and SLOMG groups (199.0 days vs 280.5 days, p = 0.463). Low baseline MG-ADL score and steroid therapy were associated with better short-term prognosis (p = 0.007 and p = 0.002, respectively). For long-term prognosis, baseline bulbar and limb involvement, time to treatment initiation, and use of immunosuppressants were significant factors (p = 0.025, p = 0.004, p = 0.025, and p < 0.0001, respectively). There were no significant differences in side effects or drug withdrawal rates between two groups.

Conclusion: This study demonstrated that AChR-ab positive VLOMG patients have a favorable prognosis and responded well to medication, with age and comorbidities showing no significant impact.

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来源期刊
CiteScore
8.30
自引率
1.70%
发文量
62
审稿时长
15 weeks
期刊介绍: Therapeutic Advances in Neurological Disorders is a peer-reviewed, open access journal delivering the highest quality articles, reviews, and scholarly comment on pioneering efforts and innovative studies across all areas of neurology. The journal has a strong clinical and pharmacological focus and is aimed at clinicians and researchers in neurology, providing a forum in print and online for publishing the highest quality articles in this area.
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