School of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy,
Respiratory Unit, San Gerardo Hospital, ASST di Monza, Monza, Italy,
Respiration. 2020;99(10):838-845. doi: 10.1159/000509556. Epub 2020 Dec 2.
The prevalence and natural history of progressive fibrosing interstitial lung diseases (PF-ILDs), and their response to commonly used treatments in real life are largely unknown.
The aim of the study was to describe the prevalence, clinical characteristics, management, and outcomes of PF-ILD patients attending 2 Italian referral centers (San Gerardo Hospital, Monza, and San Giuseppe Hospital, Milan) from January 1, 2011, to July 31, 2019.
From a cohort of non-idiopathic pulmonary fibrosis fibrosing ILD patients with at least 2-year follow-up, we selected only those with progressive disease, defined as per the INBUILD trial, collecting their demographical, clinical, and functional data.
Out of the 245 fibrosing ILD patients, 75 (31%) were classified as PF-ILDs (median age 66 years, 60% males), most frequently idiopathic non-specific interstitial pneumonia (28%), followed by connective tissue disease-associated ILD (20%), chronic hypersensitivity pneumonitis, and sarcoidosis (17% each). Most patients (81%) were categorized as PF-ILDs because of forced vital capacity (FVC) decline ≥10%, while 19% experienced a marginal FVC decline (between 5 and 10%) associated with worsening respiratory symptoms or increasing extent of fibrotic changes on high-resolution computed tomography. Disease progression occurred after a median of 18 months from ILD diagnosis. The vast majority (93%) of PF-ILD patients received prednisolone, alone (40%) or associated with steroid-sparing agents (52%), and 35% of treated patients developed treatment-related adverse events. After ILD progression, the median survival was 3 (interquartile range (IQR) 2-5) years, with a 2- and 3-year mortality rate of 4 and 20%, respectively.
In a real-life setting, approximately one-third of the fibrosing ILD patients showed a progressive course despite treatment. Studies aimed to better phenotype this subgroup of patients are needed.
进展性肺纤维化间质性肺病（PF-ILD）的患病率、自然史以及它们对常用治疗方法的反应在很大程度上尚不清楚。
本研究旨在描述 2011 年 1 月 1 日至 2019 年 7 月 31 日期间，2 家意大利转诊中心（蒙扎的圣杰尔瓦西奥医院和米兰的圣朱塞佩医院）就诊的 PF-ILD 患者的患病率、临床特征、治疗方法和结局。
我们从至少有 2 年随访的非特发性肺纤维化性间质性肺病纤维化 ILD 患者队列中，仅选择那些符合 INBUILD 试验定义的进行性疾病患者，收集他们的人口统计学、临床和功能数据。
在 245 名纤维化 ILD 患者中，75 名（31%）被归类为 PF-ILD（中位年龄 66 岁，60%为男性），最常见的是特发性非特异性间质性肺炎（28%），其次是结缔组织病相关性ILD（20%）、慢性过敏性肺炎和结节病（各占 17%）。大多数患者（81%）因用力肺活量（FVC）下降≥10%而被归类为 PF-ILD，而 19%的患者经历了 FVC 下降（在 5%至 10%之间），同时伴有呼吸症状恶化或高分辨率计算机断层扫描上纤维化程度增加。ILD 诊断后中位 18 个月疾病进展。绝大多数（93%）PF-ILD 患者接受了泼尼松龙治疗，单独使用（40%）或与类固醇维持剂联合使用（52%），35%的治疗患者发生了与治疗相关的不良反应。ILD 进展后，中位生存期为 3 年（四分位距 2-5），2 年和 3 年死亡率分别为 4%和 20%。
在现实环境中，尽管进行了治疗，但约三分之一的纤维化 ILD 患者仍表现为进行性疾病。需要进行研究以更好地对这组患者进行表型分析。
