间歇性肺部疾病的疾病轨迹 - 来自EXCITING-ILD注册表的数据
Katharina Buschulte1, Hans-Joachim Kabitz2, Lars Hagmeyer3
1Center for Interstitial and Rare Lung Diseases, Thoraxklinik, University of Heidelberg, German Center for Lung Research (DZL), Heidelberg, Germany. katharina.buschulte@med.uni-heidelberg.de.
Respiratory research
|March 6, 2024
概括
间歇性肺病 (ILD) 的进展很常见,并且与较高的死亡率有关. 关键的危险因素包括肺功能下降和老年,恶化加剧了生存率.
科学领域:
- 肺病学和呼吸系统医学
- 临床流行病学临床流行病学
- 疾病进展研究 疾病进展研究
背景情况:
- 间歇性肺部疾病 (ILD) 是一组多样化的慢性肺部疾病.
- 疾病进展,称为渐进性纤维化ILD (PF-ILD),影响高达50%的患者.
- PF-ILD与增加的死亡风险有显著的关联.
研究的目的:
- 分析各种ILD类型的疾病轨迹和进展.
- 确定与ILD进展和死亡率相关的风险因素.
- 根据ILD类型,进展和恶化来评估生存时间.
主要方法:
- 对EXCITING-ILD注册表数据的分析.
- 疾病过程的分类:显著的进展 (>10%的FVC下降),中度的进展 (5-10%的FVC下降),稳定的疾病 (<5%的FVC变化) 或改善 (≥5%的FVC增加).
- 通过FVC下降定义的PF-ILD≥10%预测在24个月内或≥1次呼吸病住院治疗.
- 考克斯的比例危险模型和用于风险因素分析的后勤回归.
- 卡普兰-梅尔曲线用于生存和进展时间的估计.
主要成果:
- 在50.6%的患者中检测到进展 (n=304).
- 异形性NSIP (iNSIP) 和异形性肺纤维化 (IPF) 显示出最短的中位时间到进展.
- 降低基线FVC和年龄较大是进展的显著预测因素.
- 更高的GAP指数和ILD恶化与更短的生存时间有关.
- IPF的存活时间显著较短,相比之下,型硬化症,CTD-ILD和HP.
结论:
- 疾病进展在所有ILD类型中普遍存在,并与死亡率增加密切相关.
- 基线FVC受损,晚年,急性恶化和呼吸系统住院是关键的风险因素.
- 早期检测进展仍然是一个挑战,需要除肺功能测试 (PFT) 外的额外临床标准.
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