Faculty of Medicine & Health Sciences, University of Antwerp (UAntwerpen), Universiteitsplein 1, 2610, Antwerpen, Belgium.
FluidDA nv, Groeningenlei 132, 2550, Kontich, Belgium.
Respir Res. 2018 Nov 6;19(1):213. doi: 10.1186/s12931-018-0918-5.
Idiopathic pulmonary fibrosis (IPF) is chronic fibrosing pneumonia with an unpredictable natural disease history. Functional respiratory imaging (FRI) has potential to better characterize this disease. The aim of this study was to identify FRI parameters, which predict FVC decline in patients with IPF.
An IPF-cohort (treated with pamrevlumab for 48 weeks) was retrospectively studied using FRI. Serial CT's were compared from 66 subjects. Post-hoc analysis was performed using FRI, FVC and mixed effects models.
Lung volumes, determined by FRI, correlated with FVC (lower lung volumes with lower FVC) (R = 0.61, p < 0.001). A negative correlation was observed between specific image based airway radius (siRADaw) at total lung capacity (TLC) and FVC (R = 0.18, p < 0.001). Changes in FVC correlated significantly with changes in lung volumes (R = 0.18, p < 0.001) and siRADaw (R = 0.15, p = 0.002) at week 24 and 48, with siRADaw being more sensitive to change than FVC. Loss in lobe volumes (R = 0.33, p < 0.001), increasing fibrotic tissue (R = 0.33, p < 0.001) and airway radius (R = 0.28, p < 0.001) at TLC correlated with changes in FVC but these changes already occur in the lower lobes when FVC is still considered normal.
This study indicates that FRI is a superior tool than FVC in capturing of early and clinically relevant, disease progression in a regional manner.
特发性肺纤维化（IPF）是一种具有不可预测自然病史的慢性纤维性肺炎。功能呼吸成像（FRI）具有更好地描述这种疾病的潜力。本研究的目的是确定 FRI 参数，这些参数可预测 IPF 患者的 FVC 下降。
使用 FRI 对 IPF 队列（接受 pamrevlumab 治疗 48 周）进行回顾性研究。对 66 例患者的连续 CT 进行比较。使用 FRI、FVC 和混合效应模型进行事后分析。
通过 FRI 确定的肺容积与 FVC 相关（较低的肺容积与较低的 FVC 相关）（R=0.61，p<0.001）。在总肺活量（TLC）处的特定基于图像的气道半径（siRADaw）与 FVC 之间观察到负相关（R=0.18，p<0.001）。FVC 的变化与肺容积（R=0.18，p<0.001）和 siRADaw（R=0.15，p=0.002）在第 24 周和第 48 周的变化显著相关，而 siRADaw 对变化更敏感比 FVC。肺叶容积的丧失（R=0.33，p<0.001）、TLC 处纤维化组织的增加（R=0.33，p<0.001）和气道半径（R=0.28，p<0.001）与 FVC 的变化相关，但这些变化在 FVC 仍被认为正常时已经发生在下叶。
这项研究表明，FRI 是一种比 FVC 更优越的工具，可更准确地以区域性方式捕获早期和临床相关的疾病进展。
