Prospective study of fibrosis in the lung endpoints (PROFILE): characteristics of an incident cohort of patients with idiopathic pulmonary fibrosis
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Published version
Author(s)
Type
Journal Article
Abstract
Background: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrotic lung disease. The PROFILE study was a prospective, observational cohort study designed to better define the natural history of IPF, understand disease biology and identify biomarkers to support disease management and enhance clinical trial design.
Methods: Individuals with an incident diagnosis of IPF were recruited between 2010 and 2017 across two co-ordinating centres in the UK. Demographics, clinical measurements and blood samples were obtained at baseline, and 1, 3, 6, 12, 24, 36 months. Disease progression events were defined as death or relative FVC decline>10% at 12 months. Survival estimates were modelled using cox proportional hazards; longitudinal lung function decline was estimated using mixed
effect models, specified with restricted cubic splines, a random intercept for participant and random effect for study visit. All models were adjusted for baseline age, sex and continuous baseline percent predicted forced vital capacity (ppFVC).
Results: A total of 632 participants were recruited, 77.1% were male and mean age at enrolment was 70.4 years (SD 8.4). Mean baseline ppFVC was 79.5% (SD 19.2), mean percent predicted DLCO (ppDLCO) was 45.7% (SD 15.1). A total of 304 (48.1%) participants met disease progression criteria at 1 year. Median survival was 3.7 years (95%CI 3.3; 4.0). More severe baseline
physiology, 12-month relative lung function decline ≥10%, older age, and short telomeres were independent risk factors for mortality. Twelve-month estimated change in ppFVC was -5.28% (95%CI -6.34; -4.22) with an average FVC decline of 186.9ml (95%CI -225.4.0; -148.5), 12-
month estimated change in ppDLCO was -3.35% (95%CI -4.30; -2.40).
Conclusion: The PROFILE cohort confirms that untreated, IPF is inexorable progressive and inevitably fatal with a poor median survival from diagnosis.
Methods: Individuals with an incident diagnosis of IPF were recruited between 2010 and 2017 across two co-ordinating centres in the UK. Demographics, clinical measurements and blood samples were obtained at baseline, and 1, 3, 6, 12, 24, 36 months. Disease progression events were defined as death or relative FVC decline>10% at 12 months. Survival estimates were modelled using cox proportional hazards; longitudinal lung function decline was estimated using mixed
effect models, specified with restricted cubic splines, a random intercept for participant and random effect for study visit. All models were adjusted for baseline age, sex and continuous baseline percent predicted forced vital capacity (ppFVC).
Results: A total of 632 participants were recruited, 77.1% were male and mean age at enrolment was 70.4 years (SD 8.4). Mean baseline ppFVC was 79.5% (SD 19.2), mean percent predicted DLCO (ppDLCO) was 45.7% (SD 15.1). A total of 304 (48.1%) participants met disease progression criteria at 1 year. Median survival was 3.7 years (95%CI 3.3; 4.0). More severe baseline
physiology, 12-month relative lung function decline ≥10%, older age, and short telomeres were independent risk factors for mortality. Twelve-month estimated change in ppFVC was -5.28% (95%CI -6.34; -4.22) with an average FVC decline of 186.9ml (95%CI -225.4.0; -148.5), 12-
month estimated change in ppDLCO was -3.35% (95%CI -4.30; -2.40).
Conclusion: The PROFILE cohort confirms that untreated, IPF is inexorable progressive and inevitably fatal with a poor median survival from diagnosis.
Date Issued
2026-01-01
Date Acceptance
2026-01-05
Citation
BMJ Open Respiratory Research, 2026, 13 (1)
ISSN
2052-4439
Publisher
BMJ Publishing Group
Journal / Book Title
BMJ Open Respiratory Research
Volume
13
Issue
1
Copyright Statement
© Author(s) (or their employer(s)) 2026. Re-use permitted under CC BY. Published by BMJ Group. This is an open access article distributed in accordance with the Creative Commons Attribution 4.0 Unported (CC BY 4.0) license
License URL
Identifier
10.1136/bmjresp-2025-003763
Publication Status
Published
Article Number
e00376
Date Publish Online
2026-01-28