Table1_Prognostic Predictive Characteristics in Patients With Fibrosing Interstitial Lung Disease: A Retrospective Cohort Study.DOCX

Background: Limited data are available regarding the entire spectrum of interstitial lung disease with a progressive fibrosing feature. We investigated the prevalence and prognostic predictive characteristics in patients with PF-ILD.
Methods: This retrospective cohort study included patients with fibrosing ILD who were investigated between 1 January 2015 and 30 April 2021. We recorded clinical features and outcomes to identify the possible risk factors for fibrosing progression as well as mortality.
Results: Of the 579 patients with fibrosing ILD, 227 (39.21%) met the criteria for progression. Clubbing of fingers [odds ratio (OR) 1.52, 95% confidence interval (CI) 1.03 to 2.24, p = 0.035] and a high-resolution computed tomography (HRCT)-documented usual interstitial pneumonia (UIP)-like fibrotic pattern (OR 1.95, 95% CI 1.33 to 2.86, p = 0.001) were risk factors for fibrosis progression. The mortality was worse in patients with PF with hypoxemia [hazard ratio (HR) 2.08, 95% CI 1.31 to 3.32, p = 0.002], in those with baseline diffusion capacity of the lung for carbon monoxide (DLCO) % predicted <50% (HR 2.25, 95% CI 1.45 to 3.50, p < 0.001), or in those with UIP-like fibrotic pattern (HR 1.68, 95% CI 1.04 to 2.71, p < 0.001).
Conclusion: Clubbing of fingers and an HRCT-documented UIP-like fibrotic pattern were more likely to be associated with progressive fibrosing with varied prevalence based on the specific diagnosis. Among patients with progressive fibrosing, those with hypoxemia, lower baseline DLCO% predicted, or UIP-like fibrotic pattern showed poor mortality.

背景：目前针对涵盖全谱系的进行性纤维化性间质性肺疾病（progressive fibrosing interstitial lung disease, PF-ILD）的相关数据仍较为有限。本研究旨在探讨PF-ILD患者的患病率及预后预测特征。
方法：本回顾性队列研究纳入了2015年1月1日至2021年4月30日期间接受评估的纤维化性间质性肺疾病患者。研究人员记录了患者的临床特征与临床结局，以明确纤维化进展及死亡的潜在危险因素。
结果：在579例纤维化性间质性肺疾病患者中，227例（39.21%）符合疾病进展的判定标准。杵状指（clubbing of fingers）[比值比（odds ratio, OR）=1.52，95%置信区间（95% confidence interval, CI）为1.03~2.24，p=0.035]及高分辨率计算机断层扫描（high-resolution computed tomography, HRCT）证实的普通型间质性肺炎（usual interstitial pneumonia, UIP）样纤维化表型[OR=1.95，95%CI为1.33~2.86，p=0.001]是纤维化进展的危险因素。合并低氧血症的进行性纤维化性间质性肺疾病患者[风险比（hazard ratio, HR）=2.08，95%CI为1.31~3.32，p=0.002]、基线肺一氧化碳弥散量占预计值百分比（diffusion capacity of the lung for carbon monoxide % predicted, DLCO% predicted）<50%的患者[HR=2.25，95%CI为1.45~3.50，p<0.001]，以及存在UIP样纤维化表型的患者[HR=1.68，95%CI为1.04~2.71，p<0.001]，其死亡风险均更高。
结论：杵状指与HRCT证实的UIP样纤维化表型更易与进行性纤维化性改变相关，且其患病率因具体诊断类型而异。在进行性纤维化性间质性肺疾病患者中，合并低氧血症、基线DLCO% predicted较低或存在UIP样纤维化表型的患者死亡风险更高。