First Department of Internal Medicine.
Department of Radiology.
Medicine (Baltimore). 2021 Apr 2;100(13):e25322. doi: 10.1097/MD.0000000000025322.
Progressive fibrosing interstitial lung disease (PF-ILD) is a progressive phenotype of fibrosing ILDs with varying definitions and elusive clinical characteristics. We aimed to clarify the clinical features and prognosis of PF-ILD cases based on the deterioration of pulmonary function. Altogether, 91 consecutive ILD patients who underwent at least 2 pulmonary function tests (PFTs) with an interval of at least 24 months, as the screening period, between January 2009 and December 2015 were retrospectively reviewed. The deterioration of forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLco) was calculated based on PFT data and screening period. The definition of PF-ILD was 1. relative decline of 10% or more in FVC per 24 months or 2. relative decline in FVC of 5% or more with decline in DLco of 15% or more per 24 months. Medical records of 34 patients with idiopathic pulmonary fibrosis (IPF), 11 patients with non-IPF, PF-ILD, and 46 patients with non-IPF, non-PF-ILD were retrospectively analyzed. Patient characteristics, pharmacologic or non-pharmacologic treatment status, and prognosis were compared between the IPF and non-IPF groups and between the non-IPF, PF-ILD and non-IPF, non-PF-ILD groups. Eleven patients (19.3%) showed a progressive phenotype in the non-IPF group. The pulmonary function data at the first PFT were worse in non-IPF, PF-ILD patients than in non-IPF, non-PF-ILD patients. There were no differences in the proportion of patients who were observed without pharmacologic treatment or of those receiving pharmacologic treatment between the non-IPF, PF-ILD and non-IPF, non-PF-ILD groups. Low %FVC at the first PFT and the usual interstitial pneumonia-like fibrotic pattern on high-resolution computed tomography were risk factors for PF-ILD in the non-IPF group. The mortality in the non-IPF, PF-ILD group was significantly worse than that of the non-IPF, non-PF-ILD group and was as poor as that of the IPF group. Multivariate logistic analysis showed that aging and low %DLco at the first PFT were risk factors for mortality within the non-IPF group. The prognosis of non-IPF, PF-ILD patients was as poor as that of IPF patients. Non-IPF, PF-ILD patients require more intensive treatment before disease progression.
进行性纤维化间质性肺病（PF-ILD）是一种纤维化间质性肺病的进行性表型，具有不同的定义和难以捉摸的临床特征。我们旨在根据肺功能恶化来阐明 PF-ILD 病例的临床特征和预后。
共回顾了 2009 年 1 月至 2015 年 12 月期间，91 例连续接受至少 2 次肺功能检查（PFT）且两次检查间隔至少 24 个月的间质性肺病患者。根据 PFT 数据和筛查期计算用力肺活量（FVC）和一氧化碳弥散量（DLco）的恶化程度。PF-ILD 的定义为：每 24 个月 FVC 相对下降 10%或更多，或每 24 个月 FVC 相对下降 5%以上，同时 DLco 下降 15%或更多。回顾性分析了 34 例特发性肺纤维化（IPF）患者、11 例非 IPF、PF-ILD 患者和 46 例非 IPF、非 PF-ILD 患者的病历。比较了 IPF 组和非 IPF 组、非 IPF、PF-ILD 组和非 IPF、非 PF-ILD 组之间的患者特征、药物或非药物治疗状况和预后。非 IPF 组中有 11 例（19.3%）表现出进行性表型。非 IPF、PF-ILD 患者首次 PFT 的肺功能数据比非 IPF、非 PF-ILD 患者差。在非 IPF、PF-ILD 组和非 IPF、非 PF-ILD 组之间，未观察到药物治疗或接受药物治疗的患者比例没有差异。首次 PFT 时低 %FVC 和高分辨率计算机断层扫描上的普通间质性肺炎样纤维化模式是非 IPF 组发生 PF-ILD 的危险因素。非 IPF、PF-ILD 组的死亡率明显高于非 IPF、非 PF-ILD 组，与 IPF 组相当。多变量逻辑分析显示，首次 PFT 时年龄较大和 %DLco 较低是非 IPF 组死亡的危险因素。非 IPF、PF-ILD 患者的预后与 IPF 患者一样差。非 IPF、PF-ILD 患者在疾病进展前需要更积极的治疗。
