Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam-S, Gyeonggi-Do, Republic of Korea.
Department of Radiology, University of Ulsan College of Medicine, Asan Medical Centre, Seoul, Republic of Korea.
Respir Res. 2021 Oct 31;22(1):282. doi: 10.1186/s12931-021-01879-6.
The progressive fibrosing (PF) phenotype of interstitial lung disease (ILD) is characterised by worsening respiratory symptoms, lung function, and extent of fibrosis on high-resolution computed tomography. We aimed to investigate the prevalence and clinical outcomes of PF-ILD in a real-world cohort and assess the prognostic significance of the PF-ILD diagnostic criteria.
Clinical data of patients with fibrosing ILD other than idiopathic pulmonary fibrosis (IPF) consecutively diagnosed at a single centre were retrospectively reviewed. A PF phenotype was defined based on the criteria used in the INBUILD trial.
The median follow-up duration was 62.7 months. Of the total of 396 patients, the mean age was 58.1 years, 39.9% were men, and rheumatoid arthritis-ILD was the most common (42.4%). A PF phenotype was identified in 135 patients (34.1%). The PF-ILD group showed lower forced vital capacity and total lung capacity (TLC) than the non-PF-ILD group. The PF-ILD group also showed poorer survival (median survival, 91.2 months vs. not reached; P < 0.001) than the non-PF-ILD group. In multivariable Cox analysis adjusted for age, DL, HRCT pattern, and specific diagnosis, PF phenotype was independent prognostic factor (hazard ratio, 3.053; P < 0.001) in patients with fibrosing ILD. Each criterion of PF-ILD showed similar survival outcomes.
Our results showed that approximately 34% of patients with non-IPF fibrosing ILD showed a progressive phenotype and a poor outcome similar to that of IPF, regardless of the diagnostic criteria used.
间质性肺疾病（ILD）的进行性纤维化（PF）表型的特征是呼吸症状、肺功能恶化，以及高分辨率计算机断层扫描显示纤维化程度增加。我们旨在调查真实世界队列中 PF-ILD 的患病率和临床结局，并评估 PF-ILD 诊断标准的预后意义。
回顾性分析在一家单中心连续诊断的除特发性肺纤维化（IPF）以外的纤维化性ILD 患者的临床数据。PF 表型根据 INBUILD 试验中使用的标准定义。
中位随访时间为 62.7 个月。在总共 396 名患者中，平均年龄为 58.1 岁，39.9%为男性，最常见的是类风湿关节炎相关ILD（42.4%）。135 名患者（34.1%）被确定为 PF 表型。PF-ILD 组的用力肺活量和肺总量（TLC）均低于非-PF-ILD 组。PF-ILD 组的生存情况也较差（中位生存时间，91.2 个月 vs. 未达到；P<0.001）。在调整年龄、DL、HRCT 模式和特定诊断的多变量 Cox 分析中，PF 表型是非 IPF 纤维化 ILD 患者的独立预后因素（风险比，3.053；P<0.001）。PF-ILD 的每个标准均显示出相似的生存结果。
我们的研究结果表明，约 34%的非 IPF 纤维化 ILD 患者表现出进行性表型和与 IPF 相似的不良结局，无论使用何种诊断标准。
