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Observational Study
. 2025 Aug;168(2):415-422.
doi: 10.1016/j.chest.2025.02.018. Epub 2025 Feb 26.

Changes in Lung Function and Mortality Risk in Patients With Idiopathic Pulmonary Fibrosis

Justin M Oldham  1 , Megan L Neely  2 , Daniel M Wojdyla  3 , Mridu Gulati  4 , Peide Li  5 , Divya C Patel  5 , Scott M Palmer  2 , Jamie L Todd  6 ; IPF-PRO Registry Investigators
Affiliations
Free article
Observational Study

Changes in Lung Function and Mortality Risk in Patients With Idiopathic Pulmonary Fibrosis

Justin M Oldham et al. Chest. 2025 Aug.
Free article

Abstract

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease associated with lung function decline and high mortality.

Research question: What are the associations between thresholds of lung function decline and the risk of mortality in patients with IPF?

Study design and methods: The Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry enrolled patients with IPF that was diagnosed or confirmed at the enrolling center within the prior 6 months. Associations between time to first decline in FVC or diffusing capacity of the lungs for carbon monoxide (Dlco) of ≥ 2% predicted, ≥ 5% predicted, and ≥ 10% predicted (and ≥ 15% predicted for Dlco) and risk of subsequent death or lung transplantation was assessed using Cox proportional hazards models with a time-dependent covariate. Models were unadjusted or adjusted for FVC and Dlco % predicted, age, sex, smoking status, BMI, antifibrotic treatment (yes or no), and oxygen use at enrollment.

Results: Among 1,001 patients, median follow-up time was 38.4 months. Significant associations were observed between all thresholds of decline in FVC and Dlco % predicted and the risk of death or lung transplantation in unadjusted and adjusted analyses. In adjusted analyses, absolute declines in FVC of ≥ 2% predicted, ≥ 5% predicted, and ≥ 10% predicted were associated with 1.8-fold, 2.3-fold, and 2.7-fold increases in the risk of subsequent death or lung transplantation, whereas absolute declines in Dlco of ≥ 2% predicted, ≥ 5% predicted, ≥ 10% predicted, and ≥ 15% predicted were associated with 2.0-fold, 1.4-fold, 1.5-fold, and 1.9-fold increases in the risk of subsequent death or lung transplantation, respectively. For Dlco, but not FVC, the increase in risk generally was greater for patients meeting a threshold based on a relative rather than an absolute decline.

Interpretation: Our results show that even small declines in FVC and Dlco % predicted inform prognosis in patients with IPF.

Clinical trial registry: ClinicalTrials.gov; No.: NCT01915511; URL: www.

Clinicaltrials: gov.

Keywords: interstitial lung disease; pulmonary fibrosis; respiratory function tests.

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Conflict of interest statement

Financial/Nonfinancial Disclosures The authors have reported to CHEST the following: J. M. O. serves as a principal investigator at a site in the IPF-PRO/ILD-PRO Registry and reports consulting fees from Boehringer Ingelheim. M. L. N., D. M. W., S. M. P., and J. L. T. are employees of the Duke Clinical Research Institute, which receives funding support from Boehringer Ingelheim Pharmaceuticals, Inc., to coordinate the IPF-PRO/ILD-PRO Registry. S. M. P. also reports research funding paid to the Duke Clinical Research Institute from Bristol Myers Squibb; royalties or licenses from UpToDate; and consulting fees from Bristol Myers Squibb, Mallinckrodt, and Sanofi. J. L. T. also reports grants from AstraZeneca and CareDx and has participated on advisory boards for Altavant, Avalyn, Natera, Sanofi, and Theravance. M. G. serves as a principal investigator for the IPF-PRO/ILD-PRO Registry and as a principal investigator for trials for Bristol Myers Squibb and Avalyn and has received consulting fees from Avalyn and the Foundation for Sarcoidosis Research. P. L. and D. C. P. are employees of Boehringer Ingelheim Pharmaceuticals, Inc.

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