渐进性肺纤维化及其对系统性硬化相关的间歇性肺病生存的影响
Kathleen Morrisroe1,2, Dylan Hansen2, Wendy Stevens2
1Department of Medicine, The University of Melbourne at St Vincent's Hospital, Melbourne, VIC, Australia.
Rheumatology (Oxford, England)
|September 19, 2023
概括
渐进性肺纤维化 (PPF) 在诊断一年内影响了近40%的系统性硬化相关的间歇性肺病 (SSc-ILD) 患者. PPF显著增加死亡风险,确定早期治疗干预的关键群体.
科学领域:
- 肺部病理学 肺部病理学
- 类风湿病学 类风湿病学
- 放射学 放射学是一门学科.
背景情况:
- 系统性硬化症 (SSc) 是一种结缔组织疾病,其特征是纤维化.
- 间歇性肺病 (ILD) 是SSc的常见和严重并发症.
- 渐进性肺纤维化 (PPF) 是SSc-ILD.中疾病发病率和死亡率的重要原因.
研究的目的:
- 在SSc-ILD.患者队列中确定PPF的发病率.
- 确定与SSc-ILD中PPF发展相关的因素.
- 评估PPF对SSc-ILD患者生存的影响.
主要方法:
- 建立了一个SSc-ILD患者的事件队列.
- PPF的定义是基于2022年美国胸腔协会的标准.
- 用了通用估计方程和加速失效时间回归来进行分析.
主要成果:
- 大约38.8%的SSc-ILD病例在12个月内发展为PPF.
- 高龄,扩散皮肤SSc (dcSSc) 和抗Scl-70抗体与PPF相关.
- PPF显著与SSc-ILD患者的死亡率增加有关 (HR 2.66).
结论:
- PPF是SSc-ILD的常见并发症,在三分之一以上的事件中发生.
- PPF的存在是SSc-ILD死亡率的强有力的预测指标.
- 患有PPF的患者可能会从早期引入免疫抑制或抗纤维治疗中受益.
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