High-Resolution CT Change over Time in Patients with Idiopathic Pulmonary Fibrosis on Antifibrotic Treatment

Abstract

Antifibrotic treatment slows down functional decline and disease progression in idiopathic pulmonary fibrosis (IPF). High-resolution computed tomography (HRCT) is useful to diagnose IPF; however, little is known about whether and to what extent HRCT changes reflect functional changes during antifibrotic therapy. The aim of this study was, therefore, to assess HRCT change over time after 1 year of treatment and to evaluate whether these changes correlate with functional decline over the same period of time. Sixty-eight IPF patients on antifibrotic treatment (i.e., pirfenidone or nintedanib) were functionally categorized as stable or progressors based on whether (or not) they had a decline in forced vital capacity (FVC) >5% predicted/year, and their HRCT were scored blindly and independently by two expert thoracic radiologists at treatment initiation (HRCT1) and after 1 year of treatment (HRCT2). Ground glass opacities (Alveolar Score, AS), reticulations (Interstitial Score, IS) and honeycombing (HC) were quantified and correlated with FVC decline between HRCT1 and HRCT2. At treatment initiation, HRCT scores were similar in both stable patients and progressors. After one year of treatment, in the entire population, AS and HC increased significantly, while IS did not. However, when stratified by the rate of functional decline, in stable patients, HC increased significantly while AS and IS did not. On the other hand, among progressors AS and HC increased significantly whereas IS did not. In the entire population, the combined score of fibrosis (IS + HC) correlated significantly with FVC decline. In conclusion, IPF patients on antifibrotic treatment exhibit different patterns of HRCT change over time based on their rate of functional decline. HRCT data should be integrated to lung function data when assessing response to antifibrotic treatment in patients with IPF.

Prognostic role of MUC5B rs35705950 genotype in patients with idiopathic pulmonary fibrosis (IPF) on antifibrotic treatment

“…or stable (< 5%pred. ), as previously reported [ 15 , 16 ]. Improvement of FVC was expressed as negative value.…”

[18F]FMISO PET/CT imaging of hypoxia as a non-invasive biomarker of disease progression and therapy efficacy in a preclinical model of pulmonary fibrosis: comparison with the [18F]FDG PET/CT approach

“…Nevertheless, our preclinical study highlighted the fact that baseline CT quantification correlated with pirfenidone efficacy in accordance with a clinical study by Higo et al [28] which demonstrates that an early deterioration of HRCT was significantly more observed in the progressive versus improved/stable IPF patients under pirfenidone treatment. Similarly, increased honeycombing and ground glass opacities on HRCT were shown to be associated with progressive IPF patients under pirfenidone [29]. Despite such promising preclinical and clinical results, HRCT quantification is not yet integrated in a routine clinic.…”

Effective block by pirfenidone, an antifibrotic pyridone compound (5-methyl-1-phenylpyridin-2[H-1]-one), on hyperpolarization-activated cation current: An additional but distinctive target

“…This compound is a new anti-fibrotic drug approved by the U.S. Food and Drug Administration for idiopathic pulmonary fibrosis. Accumulating evidence has revealed the efficacy of PFD either in treating idiopathic pulmonary fibrosis (Balestro et al, 2019;Saito et al, 2019), in interstitial lung disease (Collins and Raghu, 2019), or in non-small cell lung cancer (Kanayama et al, 2020;Krämer et al, 2020). Recently, through its anti-inflammatory and anti-oxidant effects, namely by inhibiting IL-1β and IL-4, PFD has been included in a clinical trial for the treatment of coronavirus disease (COVID-19) (NCT04282902, 2020; Rosa and Santos, 2020).…”