系统性硬化症的不同定义的一致性和预后相关性 间歇性肺部疾病进展
Enrico De Lorenzis1,2, Francesco Del Galdo2, Gerlando Natalello1
1Division of Rheumatology, Department of Geriatrics, Orthopedics and Rheumatology.
系统性硬化症相关的间歇性肺病 (SSc-ILD) 的进展定义在准确性上有所不同. 四个定义显示出不同的预后值,影响了患者的分类和结果,特别是在特定的临床子组.
科学领域:
- 类风湿病学 类风湿病学
- 肺部病理学 肺部病理学
- 临床试验 临床试验
背景情况:
- 系统性硬化症相关的间歇性肺病 (SSc-ILD) 的进展和预后各不相同.
- 现有的ILD进展的定义可能会被SSc患者的肺功能和症状变化所混.
研究的目的:
- 评估四个不同的SSc-ILD进展定义的一致性和预后意义.
- 为了比较与SSc-ILD患者不同进展标准相关的死亡风险.
主要方法:
- 在245名患者中对24个月的SSc-ILD进展进行了评估.
- 使用四个定义,比较进展者和非进展者之间的60个月死亡风险:临床上最少重要的FVC恶化,EUSTAR,OMERACT和Erice标准.
主要成果:
- 发生了26起与SSc相关的死亡.
- 通过临床上最少重要的FVC恶化,OMERACT和Erice标准的进展与死亡率的增加有关.
- 根据疾病持续时间,功能障碍和肺动脉静脉压等因素,预后值在各小组之间有显著差异.
结论:
- 评估的四个SSc-ILD进展定义是不可互换的.
- 分类和预后值不同,特别是在特定的临床患者群体中.
- 这凸显了在SSc-ILD研究和临床实践中仔细选择进展标准的必要性.
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