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全身型重症肌无力的预后预测因素:一项前瞻性观察研究。

Outcome Predictors of Generalized Myasthenia Gravis: A Prospective Observational Study.

作者信息

Kalita Jayantee, Gutti Nagendra B, Ahamed Faim

机构信息

Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

出版信息

Ann Indian Acad Neurol. 2025 Jan 1;28(1):58-65. doi: 10.4103/aian.aian_386_24. Epub 2025 Jan 24.

Abstract

BACKGROUND AND OBJECTIVES

There is paucity of studies on long-term remission of autoimmune generalized myasthenia gravis (MG) from Southeast Asia. We report the outcome predictors of generalized MG and also evaluate the influence of high- versus low-dose prednisolone and prednisolone with or without azathioprine (AZA).

METHODS

Fifty-seven patients with generalized MG were included, who completed 2 years of follow-up. Demographic information, comorbidities, Myasthenia Gravis Foundation of America (MGFA) class at baseline and follow-up, acetylcholine receptor (AChR) and muscle-specific kinase antibodies, decremental response, thymectomy, and treatments were recorded. Maximum doses of prednisolone, AZA, and acetylcholinesterase inhibitors were noted. The predictors of MGFA 0 at 3 and 6 months and minimal manifestation (MM) status at 2 years were evaluated.

RESULTS

MGFA 0 was achieved by 27 (47.4%) patients at 3 months, 35 (61.4%) patients at 6 months, and 46 (80.7%) patients at 12 months. At 2 years, 48 (84.2%) patients achieved the MM status and none achieved complete stable or pharmacologic remission. On multivariate analysis, AChR antibody titer (adjusted odds ratio [AOR] 1.08, 95% confidence interval [CI] 1.006-1.167; P = 0.03) and MG activity of daily living (MGADL) at 6 months (AOR 1.28, 95% CI 1.066-1.558; P = 0.01) predicted the MM status. Maximum dose of prednisolone and adjunctive AZA did not predict the MM status.

CONCLUSIONS

About 84.2% of patients with generalized MG, especially those with a low AChR antibody titer and MGADL < 4 at 6 months, achieved the MM status at 2 years.

摘要

背景与目的

东南亚地区关于自身免疫性全身型重症肌无力(MG)长期缓解的研究较少。我们报告了全身型MG的预后预测因素,并评估了高剂量与低剂量泼尼松龙以及泼尼松龙联合或不联合硫唑嘌呤(AZA)的影响。

方法

纳入57例全身型MG患者,他们完成了2年的随访。记录人口统计学信息、合并症、基线和随访时的美国重症肌无力基金会(MGFA)分级、乙酰胆碱受体(AChR)和肌肉特异性激酶抗体、递减反应、胸腺切除术及治疗情况。记录泼尼松龙、AZA和乙酰胆碱酯酶抑制剂的最大剂量。评估3个月和6个月时达到MGFA 0级以及2年时达到最小表现(MM)状态的预测因素。

结果

3个月时27例(47.4%)患者达到MGFA 0级,6个月时35例(61.4%)患者达到,12个月时46例(80.7%)患者达到。2年时,48例(84.2%)患者达到MM状态,无患者达到完全稳定或药物缓解。多因素分析显示,AChR抗体滴度(调整优势比[AOR] 1.08,95%置信区间[CI] 1.006 - 1.167;P = 0.03)和6个月时的MG日常生活活动能力(MGADL)(AOR 1.28,95% CI 1.066 - 1.558;P = 0.01)可预测MM状态。泼尼松龙的最大剂量和辅助使用AZA不能预测MM状态。

结论

约84.2%的全身型MG患者,尤其是AChR抗体滴度低且6个月时MGADL < 4的患者,2年时达到MM状态。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9186/11892981/5d9fd05a0176/AIAN-28-58-g001.jpg

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