Abstract
BackgroundIdiopathic 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.MethodsAn 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.ResultsLung volumes, determined by FRI, correlated with FVC (lower lung volumes with lower FVC) (R2 = 0.61, p < 0.001). A negative correlation was observed between specific image based airway radius (siRADaw) at total lung capacity (TLC) and FVC (R2 = 0.18, p < 0.001). Changes in FVC correlated significantly with changes in lung volumes (R2 = 0.18, p < 0.001) and siRADaw (R2 = 0.15, p = 0.002) at week 24 and 48, with siRADaw being more sensitive to change than FVC. Loss in lobe volumes (R2 = 0.33, p < 0.001), increasing fibrotic tissue (R2 = 0.33, p < 0.001) and airway radius (R2 = 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.ConclusionThis study indicates that FRI is a superior tool than FVC in capturing of early and clinically relevant, disease progression in a regional manner.
Highlights
Idiopathic pulmonary fibrosis (IPF) is chronic fibrosing pneumonia with an unpredictable natural disease history
In the Phase 2 study, conducted by FibroGen, Inc., diagnosis of IPF was based on a usual interstitial pneumonia (UIP)-pattern on high resolution computed tomography (HRCT) or a possible UIP-pattern with a UIP-pattern on surgical lung biopsy, as per applicable diagnostic guidelines at the time (2011) [2]
Patients had been diagnosed with IPF within 5 years of trial inclusion with Forced Vital Capacity (FVC) ≥ 45% predicted and DLCO ≥ 30% predicted and participants had to show disease progression in the last year (FVC decline ≥10%, HRCT worsening, and/or other objective changes)
Summary
Idiopathic pulmonary fibrosis (IPF) is chronic fibrosing pneumonia with an unpredictable natural disease history. The aim of this study was to identify FRI parameters, which predict FVC decline in patients with IPF. Idiopathic pulmonary fibrosis (IPF) is a fatal, chronic fibrosing interstitial pneumonia with a variable and unpredictable natural history [1,2,3]. FVC best predicts disease progression and mortality [9, 10] It serves as a primary endpoint in IPF, it’s not a proven surrogate for mortality [11, 12]. A 2–6% change in predicted FVC has been proposed as the minimal clinical important difference [9], and where 10% decline in absolute FVC correlated well with mortality [10]. In future studies, where patients are treated with (combination of) antifibrotic drugs, its use as a clinical endpoint is less compelling since FVC decline is impacted by this therapy [14,15,16]
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