TY - JOUR T1 -进行性纤维化间质性肺病:临床队列研究(进步)摩根富林明-欧洲呼吸杂志》乔和J - 10.1183/13993003.02718 -2020欧元六世- 57 - 2 SP - 2002718 AU -纳赛尔,Mouhamad盟——Larrieu苏菲盟——Si-Mohamed萨利姆AU -艾哈迈德,凯盟——Boussel卢AU -布莱卫,玛丽盟——Chalabreysse劳拉盟——法布尔,席琳盟——品牌、赛盟——陶醉,迪迪埃盟——Thivolet-Bejui,弗朗索瓦丝AU - Traclet,朱莉AU - Zeghmar,塞布丽娜盟——Maucort-Boulch戴尔芬盟——Cottin在慢性纤维化间质性肺疾病(ILD)患者中，可能会出现进行性纤维化表型(PF-ILD)，但在临床试验之外缺乏这一人群的频率和特征的信息。我们在一个真实的单中心临床队列中评估了除特发性肺纤维化(IPF)外的PF-ILD患者的特征和预后。回顾性检查所有连续成年纤维化间质性肺病患者(2010-2017年)的档案，以确定高分辨率计算机断层扫描纤维化≥10%的预定义标准，并在重叠窗口2年期间疾病进展。基线定义为确定疾病进展的日期。接受尼intedanib或吡非尼酮的患者从生存和进展分析中被删除。共筛查了1395例患者;617人有IPF以外的ILD或合并肺纤维化和肺气肿，168人有进行性纤维化表型。165例可评估患者中位年龄为61岁;57%为女性。基线平均用力肺活量(FVC)预测值为74±22%。 Median duration of follow-up was 46.2 months. Annualised FVC decline during the first year was estimated at 136±328 mL using a linear mixed model. Overall survival was 83% at 3 years and 72% at 5 years. Using multivariate Cox regression analysis, mortality was significantly associated with relative FVC decline ≥10% in the previous 24 months (p<0.05), age ≥50 years (p<0.01) and diagnosis subgroup (p<0.01).In this cohort of patients with PF-ILD not receiving antifibrotic therapy, the disease followed a course characterised by continued decline in lung function, which predicted mortality.In a real-world clinical cohort (PROGRESS), progressive fibrosing interstitial lung disease was characterised by continued lung function decline. Lung function decline, age and underlying diagnosis subgroup predicted mortality. https://bit.ly/2EB3OpF ER -