Observational studyBMJ open respiratory research2026
Prospective study of fibrosis in the lung endpoints (PROFILE): characteristics of an incident cohort of patients with idiopathic pulmonary fibrosis.
Observational study in BMJ open respiratory research, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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13 authors.
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Abstract
backgroundIdiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrotic lung disease. Prospective study of fibrosis in the lung endpoints (PROFILE) 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.
methodsIndividuals 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 and 36 months. Disease progression events were defined as death or relative forced vital capacity (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 FVC (ppFVC).
resultsA 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), and mean percent predicted DL
conclusionThe PROFILE cohort confirms that untreated IPF is inexorably progressive and inevitably fatal with a poor median survival from diagnosis.
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