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

Elisabetta Balestro,Marina Saetta,Donato Lacedonia,Erica Bazzan,Linda Mazzai,Paolo Spagnolo,Chiara Giraudo,Graziella Turato,Davide Biondini,Elisabetta Cocconcelli,Mariaenrica Tinè,Giulia Rizzon,Roberta Polverosi,Manuel G Cosio,Sara Lococo

doi:10.3390/jcm8091469

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.

Highlights

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial lung disease (ILD) of unknown etiology that leads to respiratory failure and death within 3–5 years from diagnosis if untreated [1]
Lung function tests have been used for monitoring IPF and forced vital capacity (FVC) decline is widely accepted as a surrogate of disease progression, and possibly mortality, in IPF [5,6]
We aimed to evaluate whether and to what extent High-Resolution Computed Tomography (HRCT) abnormalities—as assessed by semiquantitative visual score—change after 1 year of antifibrotic treatment and how these changes correlate with different functional disease trajectories in patients with IPF

Summary

Introduction

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial lung disease (ILD) of unknown etiology that leads to respiratory failure and death within 3–5 years from diagnosis if untreated [1]. Disease extent on HRCT (i.e., extent of reticular and honeycombing change) correlates with disease severity and prognosis in untreated patients with IPF [7,8,9]. Inter-observer variation for the visual estimation of the extent of disease pattern is unavoidable but can be mitigated with a continuous learning method to reach a consensus [12,13]. With this background, we aimed to evaluate whether and to what extent HRCT abnormalities—as assessed by semiquantitative visual score—change after 1 year of antifibrotic treatment and how these changes correlate with different functional disease trajectories (i.e., stable patients vs progressors) in patients with IPF We aimed to evaluate whether and to what extent HRCT abnormalities—as assessed by semiquantitative visual score—change after 1 year of antifibrotic treatment and how these changes correlate with different functional disease trajectories (i.e., stable patients vs. progressors) in patients with IPF

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Conclusion