TY - T1的进步fibrosing间质性肺疾病: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AU - 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文森特Y1 - 2021/02/01 UR - //www.qdcxjkg.com/content/57/2/2002718.abstract N2患者慢性fibrosing间质性肺病(ILD)累进fibrosing表型(PF-ILD)可能发展,但信息的频率和特征这一人群外缺乏临床试验。我们评估的特点和结果PF-ILD患者除了特发性肺纤维化(IPF)在真实的,只有临床队列。所有连续的文件成人患者fibrosing ILD(2010 - 2017)的预定义的标准进行回顾性分析纤维化≥10%高分辨率计算机断层扫描和进步的疾病在重叠窗口2年。基线定义为疾病进展被确认的日期。病人接受nintedanib或未通过审查pirfenidone生存和发展分析。总共1395名患者筛选;617年ILD除了IPF或合并肺纤维化和肺气肿,和168年进步fibrosing表型。165年可评价的患者,平均年龄是61岁;57%是女性。基线是指用力肺活量(FVC)是74±22%的预测。平均随访时间为46.2个月。年FVC下降在第一年被估计为136±328毫升使用线性混合模型。 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 -