Kim John S, Pugashetti Janelle, Ma Shwu-Fan, Huang Yong, Podolanczuk Anna J, Lynch David A, Oh Andrea, Mychaleckyj Josyf, Manichaikul Ani, Rich Stephen, Chua Felix, Adams Traci M, Flaherty Kevin R, Lee Joyce S, Lasky Joseph A, Raghu Ganesh, Murray Susan, Spino Catherine, Martinez Fernando J, Noth Imre, Humphries Stephen M, Adegunsoye Ayodeji, Molyneaux Philip L, Oldham Justin M, Newton Chad A
Department of Medicine, University of Virginia School of Medicine, Charlottesville, Virginia, USA
Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA.
Thorax. 2025 Jun 8. doi: 10.1136/thorax-2024-222149.
Prior work suggests different interstitial lung diseases (ILDs) that share the radiological usual interstitial pneumonia (UIP) pattern have an overall worse prognosis. However, epidemiological data with longitudinal sampling and replication remains lacking.
Data was used from the Pulmonary Fibrosis Foundation Patient Registry (PFF-PR) (n=932) and a meta-cohort of ILD research studies (n=1579). Linear mixed-effects models and Cox proportional hazard models were used to determine forced vital capacity (FVC) slopes and 5-year transplant-free survival, respectively, by ILD diagnosis and UIP radiological pattern. Secondarily, we examined FVC and survival by diagnosis and radiological fibrosis quantified by data-driven texture analysis (DTA) in the PFF-PR. Models were adjusted for age, sex, smoking and antifibrotic and immunosuppression medication use.
The proportions of idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis (FHP) and connective tissue disease (CTD)-ILD were the following for PFF-PR (70%, 11%, 19%) and meta-cohort (21%, 32%, 47%). In the PFF-PR, CTD-ILD with UIP CT pattern was associated with slower FVC decline (-34.4 mL/year) compared with IPF (-158.4 mL/year) and longer transplant-free survival (HR 0.50, 95% CI 0.29 to 0.85). This was replicated in the meta cohort for FVC (-53.1 vs -185.9 mL/year, p<0.0001) and survival (HR 0.38, 95% CI 0.27 to 0.53). A similar pattern was seen using DTA to objectively categorise patients into higher and lower radiological fibrosis. Between IPF and FHP-UIP, FVC decline was not significantly different in the PFF-PR (-203.4 vs -158.4 mL/year, p=0.58) and meta-cohort (-124.0 vs -185.9 mL/year, p=0.25).
Even in the presence of a UIP CT pattern, there may still be differences in lung function over time and survival, particularly for CTD-ILD.
先前的研究表明，具有放射学上普通型间质性肺炎（UIP）模式的不同间质性肺疾病（ILD）总体预后较差。然而，缺乏纵向抽样和重复验证的流行病学数据。
使用来自肺纤维化基金会患者登记处（PFF-PR）（n = 932）和ILD研究的荟萃队列（n = 1579）的数据。分别使用线性混合效应模型和Cox比例风险模型，根据ILD诊断和UIP放射学模式确定用力肺活量（FVC）斜率和5年无移植生存率。其次，我们在PFF-PR中通过诊断和数据驱动纹理分析（DTA）量化的放射学纤维化来检查FVC和生存率。模型根据年龄、性别、吸烟情况以及抗纤维化和免疫抑制药物的使用情况进行了调整。
PFF-PR中特发性肺纤维化（IPF）、纤维化性过敏性肺炎（FHP）和结缔组织病（CTD）-ILD的比例分别为70%、11%、19%，荟萃队列中的比例分别为21%、32%、47%。在PFF-PR中，具有UIP CT模式的CTD-ILD与IPF（-158.4 mL/年）相比，FVC下降较慢（-34.4 mL/年），无移植生存期更长（HR 0.50，95%CI 0.29至0.85）。这在荟萃队列中关于FVC（-53.1对-185.9 mL/年，p<0.0001）和生存率（HR 0.38，95%CI 0.27至0.53）方面得到了重复验证。使用DTA将患者客观分类为放射学纤维化程度较高和较低时，也观察到了类似模式。在PFF-PR中，IPF和FHP-UIP之间的FVC下降没有显著差异（-203.4对-158.4 mL/年，p = 0.58），在荟萃队列中也是如此（-124.0对-185.9 mL/年，p = 0.25）。
即使存在UIP CT模式，随着时间推移肺功能和生存率仍可能存在差异，尤其是对于CTD-ILD。
