关于在多发性硬化症中独立于复发活动的进展的统一定义
Emilio Portaccio1, Matteo Betti1, Ermelinda De Meo1
1Department of NEUROFARBA, University of Florence, Italy.
Neurology
|September 22, 2025
概括
进展独立于复发活动 (PIRA) 显著影响复发性多发性硬化症 (MS) 的残疾. 结合流动基线,特定复发排除和24周确认的定义最能预测长期残疾.
科学领域:
- 神经学 神经学
- 临床研究 临床研究
- 多发性硬化症的治疗方法
背景情况:
- 复发活动独立的进展 (PIRA) 是复发性多发性硬化症 (MS) 患者残疾累积的关键因素.
- 准确的PIRA识别和定义对于预测MS中长期残疾结果至关重要.
- 现有的PIRA定义各不相同,需要对其预测性能的评估.
研究的目的:
- 根据残疾确认和复发排除的不同标准,评估PIRA的18个不同的定义.
- 确定哪个PIRA定义最准确地预测了达到显著残疾水平的风险 (EDSS = 6.0).
- 确定一个可行的和可预测的PIRA定义,用于临床实践和研究.
主要方法:
- 从意大利多发性硬化和相关疾病登记册中对30203名复发性多发性硬化患者进行了回顾性分析.
- 纳入标准:在2000年1月1日或之后的第一次访问,至少有三次访问与扩展残疾状况量表 (EDSS) 评估,以及至少5年的随访.
- 测试了18个PIRA定义,不同的基线标准,确认窗口 (24或48周),复发排除期 (90或180天) 和没有复发 (ABS).
主要成果:
- 在11.3年的随访期间,PIRA患病率在各定义之间从38.8%到74.1%不等.
- 15%的患者达到EDSS分数为6.0. 这些患者的EDSS分数为6.0.
- 90天复发排除标准 (90d) 产生了更高的敏感性 (66.7%-69.4%),而没有复发 (ABS) 标准提高了特异性 (55.3%-62.2%).
结论:
- 一个包含漫游基线的PIRA定义,排除在事件发生前90天和30天内复发的情况,以及24周的确认证明了最好的预测价值.
- 这种优化的PIRA定义为复发性MS的长期残疾提供了高可行性和预测准确性.
- 这些发现支持在MS进展的常规临床监测和科学研究中采用这种精细的PIRA定义.
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