Computed tomography patterns predict clinical course of idiopathic pulmonary fibrosis

Byoung Soo Kwon,Hee Sang Hwang,Jooae Choe,Kyung Hyun Do,Jin Woo Song,Eun Jin Chae

doi:10.1186/s12931-020-01562-2

Abstract

BackgroundA new clinical guideline for idiopathic pulmonary fibrosis (IPF) uses high-resolution computed tomography (HRCT) patterns for diagnostic purposes. However, it is unknown how they relate to the IPF clinical course. We aimed to investigate whether HRCT patterns could be used to predict lung function changes and survival in patients with IPF.MethodsClinical data were retrospectively reviewed in 337 patients with IPF (all biopsy-proven cases). HRCT patterns were classified according to the 2018 IPF diagnostic criteria.ResultsThe median follow-up was 46.9 months. The mean age was 62.5 years, and 74.2% were men. Among the HRCT patterns, usual interstitial pneumonia (UIP), probable UIP, indeterminate for UIP, and an alternative diagnosis were identified in 163 (48.4%), 110 (32.6%), 33 (9.8%), and 31 (9.2%) patients, respectively. The indeterminate for UIP group showed higher lung function and exercise capacity and better prognosis than the other groups. They also had a lesser decline in lung function than the other groups during follow-up. In the multivariate Cox analysis, which was adjusted by age, smoking status, lung function, exercise capacity, and use of antifibrotic agents, indeterminate for UIP pattern was found to be an independent prognostic factor (hazard ratio 0.559, 95% confidence interval 0.335–0.933, P = 0.026). However, the probable UIP group had similar lung function changes and prognosis when compared the UIP group.ConclusionsOur results suggest that indeterminate for UIP pattern on HRCT may predict a more favorable clinical course in patients with IPF, supporting the validity of the new IPF diagnostic guidelines.

Highlights

Idiopathic pulmonary fibrosis (IPF) is the most common type of idiopathic interstitial pneumonia
Acardu et al conducted a retrospective analysis of 350 patients with idiopathic pulmonary fibrosis (IPF) and reported that a consistent usual interstitial pneumonia (UIP) pattern was not associated with poorer survival than a possible or inconsistent pattern on high-resolution computed tomography (HRCT) when adjusted by age, sex, and lung function [7]
Interobserver agreement according to the HRCT patterns was the highest in UIP pattern (κ = 0.62), followed by indeterminate for UIP pattern (κ = 0.47), probable UIP pattern (κ = 0.44), and alternative diagnosis (κ = 0.36)

Summary

Introduction

Idiopathic pulmonary fibrosis (IPF) is the most common type of idiopathic interstitial pneumonia. Acardu et al conducted a retrospective analysis of 350 patients with IPF and reported that a consistent UIP pattern was not associated with poorer survival than a possible or inconsistent pattern on HRCT when adjusted by age, sex, and lung function [7]. These contradictory findings may be due to differences in baseline demographic features and disease severity between studies.

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