Computed tomography patterns predict clinical course of idiopathic pulmonary fibrosis

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RESEARCH

Computed tomography patterns predict clinical course of idiopathic pulmonary fibrosis Byoung Soo Kwon1†, Jooae Choe2†, Kyung Hyun Do2, Hee Sang Hwang3, Eun Jin Chae2† and Jin Woo Song4*† 

Abstract  Background:  A 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. Methods:  Clinical data were retrospectively reviewed in 337 patients with IPF (all biopsy-proven cases). HRCT patterns were classified according to the 2018 IPF diagnostic criteria. Results:  The 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. Conclusions:  Our 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. Keywords:  Idiopathic pulmonary fibrosis, Guideline, Survival, Respiratory function test Introduction Idiopathic pulmonary fibrosis (IPF) is the most common type of idiopathic interstitial pneumonia. It has the worst prognosis with a median survival time of 3  years [1]. The clinical course of IPF is variable [2], and predicting *Correspondence: [email protected] † Byoung Soo Kwon and Jooae Choe equally contributed to this work as first authors † Eun Jin Chae and Jin Woo Song contributed to this work as corresponding authors 4 Department of Pulmonology and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Centre, 88 Olympic‑ro 43‑gil, Songpa‑gu, Seoul 05505, South Korea Full list of author information is available at the end of the article

its prognosis is difficult. Previous studies have reported that baseline and change in lung function over time and exercise capacity are associated with poor prognosis in IPF [3–5]. Although high-resolution computed tomography (HRCT) is a pivotal modality for diagnosing IPF, it is unclear if it can be used for predicting the clinical course of IPF; previous studies on the association betw