Dong Xiao, Chen Daji, Wan Linlin, Peng Linliu, Chen Zhao, Ouyang Riwei, Long Xiafei, Du Kefang, Wu Xiaokang, Xiao Xinying, He Ruqing, Qiu Rong, Tang Beisha, Jiang Hong
Department of Neurology, Xiangya Hospital, Central South University, Changsha, 410008, China.
Key Laboratory of Hunan Province in Neurodegenerative Disorders, Central South University, Changsha, 410008, China.
J Neurol. 2025 Jun 18;272(7):467. doi: 10.1007/s00415-025-13204-y.
Multiple-system atrophy is a rapidly progressive neurodegenerative disease with incomplete survival data, limiting the understanding of long-term outcomes. This study aimed to investigate a comprehensive data including survival time and prognostic factors.
Individual patient data were pooled from studies reporting Kaplan-Meier curves, and then, survival curves were generated. The pooled median survival times were derived using weighted median of medians approach and hazard ratios of risk factors were analyzed using either fixed- or random-effects model.
37 studies involving 6145 patients were included. The median survival time for MSA patients was 8.23 years (95% CI 8.02-8.56) based on reconstructed individual patient data. The pooled weighted median time was 8.0 years (95% CI 7.51-9.0). The following variables were found as unfavorable prognostic factors (hazard ratio with 95% CI are shown): age at onset (1.02, 1.01-1.03), poor levodopa response (1.55, 1.14-2.11), parkinsonism onset (1.30, 1.05-1.62), falls (1.84, 1.4-2.4), dysautonomia onset (1.48, 1.16-1.9), autonomic failure (2.52, 1.42-4.48), orthostatism hypotension (1.39, 1.16-1.66), bladder catheterization (1.81, 1.41-2.31), and stridor (1.5, 1.12-2.02).
Survival time in MSA was evaluated using multiple methodological approaches, revealing a median survival of 8.0 years, and clinical variables like early autonomic failure and frequently falls were identified as predictors of poor survival outcomes.
多系统萎缩是一种快速进展的神经退行性疾病，生存数据不完整，限制了对长期预后的了解。本研究旨在调查包括生存时间和预后因素在内的综合数据。
从报告Kaplan-Meier曲线的研究中汇总个体患者数据，然后生成生存曲线。使用中位数的加权中位数方法得出汇总的中位生存时间，并使用固定效应或随机效应模型分析危险因素的风险比。
纳入了37项研究，共6145例患者。根据重建的个体患者数据，多系统萎缩患者的中位生存时间为8.23年（95%置信区间8.02-8.56）。汇总的加权中位时间为8.0年（95%置信区间7.51-9.0）。发现以下变量为不良预后因素（显示风险比及95%置信区间）：发病年龄（1.02，1.01-1.03）、左旋多巴反应差（1.55，1.14-2.11）、帕金森综合征起病（1.30，1.05-1.62）、跌倒（1.84，1.4-2.4）、自主神经功能障碍起病（1.48，1.16-1.9）、自主神经衰竭（2.52，1.42-4.48）、体位性低血压（1.39，1.16-1.66）、膀胱插管（1.81，1.41-2.31）和喘鸣（1.5，1.12-2.02）。
采用多种方法评估了多系统萎缩的生存时间，中位生存时间为8.0年，并确定早期自主神经衰竭和频繁跌倒等临床变量是生存预后不良的预测因素。
