Cited by 273Interstitial Lung Diseases and Idiopathic Pulmonary FibrosisOccupational and environmental lung diseasesChronic Obstructive Pulmonary Disease (COPD) Research

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An epithelial biomarker signature for idiopathic pulmonary fibrosis: an analysis from the multicentre PROFILE cohort study

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Maher, Toby M, Oballa, Eunice, Simpson, Juliet K, Porte, Joanne, Habgood, Anthony, Fahy, William A, Flynn, Aiden, Molyneaux, Philip L, Braybrooke, Rebecca, Divyateja, Hrushikesh, Parfrey, Helen, Rassl, Doris, Russell, Anne-Marie, Saini, Gauri, Renzoni, Elisabetta A, Duggan, Anne-Marie, Hubbard, Richard, Wells, Athol U, Lukey, Pauline T, Marshall, Richard P, Jenkins, R Gisli·2017·The Lancet Respiratory Medicine

DOI 10.1016/s2213-2600(17)30430-7

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Abstract

Background Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal disorder with a variable disease trajectory. The aim of this study was to assess potential biomarkers to predict outcomes for people with IPF.Methods PROFILE is a large prospective longitudinal cohort of treatment-naive patients with IPF. We adopted a two-stage discovery and validation design using patients from the PROFILE cohort. For the discovery analysis, we examined 106 patients and 50 age and sex matched healthy controls from Nottingham University Hospitals NHS Trust and the Royal Brompton Hospital. We did an unbiased, multiplex immunoassay assessment of 123 biomarkers. We further investigated promising novel markers by immunohistochemical assessment of IPF lung tissue. In the validation analysis, we examined samples from 206 people with IPF from among the remaining 212 patients recruited to PROFILE Central England. We used the samples to attempt to replicate the biomarkers identified from the discovery analysis by use of independent immunoassays for each biomarker. We investigated the predictive power of the selected biomarkers to identify individuals with IPF who were at risk of progression or death. The PROFILE studies are registered on ClinicalTrials.gov, numbers NCT01134822 (PROFILE Central England) and NCT01110694 (PROFILE Royal Brompton Hospital).Findings In the discovery analysis, we identified four serum biomarkers (surfactant protein D, matrix metalloproteinase 7, CA19-9, and CA-125) that were suitable for replication. Histological assessment of CA19-9 and CA-125 suggested that these proteins were markers of epithelial damage. Replication analysis showed that baseline values of surfactant protein D (46·6 ng/mL vs 34·6 ng/mL, p=0·0018) and CA19-9 (53·7 U/mL vs 22·2 U/mL; p<0·0001) were significantly higher in patients with progressive disease than in patients with stable disease, and rising concentrations of CA-125 over 3 months were associated with increased risk of mortality (HR 2·542, 95% CI 1·493-4·328, p=0·00059).Interpretation We have identified serum proteins secreted from metaplastic epithelium that can be used to predict disease progression and death in IPF.Funding GlaxoSmithKline R&D and the UK Medical Research Council.

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Additional file 5 of Integrative multi-omics analysis reveals novel idiopathic pulmonary fibrosis endotypes associated with disease progression
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Additional file 2 of Integrative multi-omics analysis reveals novel idiopathic pulmonary fibrosis endotypes associated with disease progression
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Additional file 1 of Integrative multi-omics analysis reveals novel idiopathic pulmonary fibrosis endotypes associated with disease progression
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Additional file 2 of Clustering of lung diseases in the family of interstitial lung disease patients
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Additional file 1 of Serum surfactant protein D as a predictive biomarker for the efficacy of pirfenidone in patients with idiopathic pulmonary fibrosis: a post-hoc analysis of the phase 3 trial in Japan
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Additional file 1 of Cutaneous T-cell-attracting chemokine as a novel biomarker for predicting prognosis of idiopathic pulmonary fibrosis: a prospective observational study
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Additional file 2 of NHLRC2 expression is increased in idiopathic pulmonary fibrosis
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Additional file 3 of Serum surfactant protein D as a predictive biomarker for the efficacy of pirfenidone in patients with idiopathic pulmonary fibrosis: a post-hoc analysis of the phase 3 trial in Japan
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Additional file 3 of Integrative multi-omics analysis reveals novel idiopathic pulmonary fibrosis endotypes associated with disease progression
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Additional file 1 of Usefulness of FAPα assessment in bronchoalveolar lavage as a marker of fibrogenesis: results of a preclinical study and first report in patients with idiopathic pulmonary fibrosis
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Additional file 1 of NHLRC2 expression is increased in idiopathic pulmonary fibrosis
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Additional file 2 of Genetic association between smoking and DLCO in idiopathic pulmonary fibrosis patients
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Additional file 2 of Serum surfactant protein D as a predictive biomarker for the efficacy of pirfenidone in patients with idiopathic pulmonary fibrosis: a post-hoc analysis of the phase 3 trial in Japan
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Authors: Maher, T. M., Oballa, E., Simpson, J. K., Porte, J., Habgood, A., Fahy, W. A., Flynn, A., Molyneaux, P. L., Braybrooke, R., Divyateja, H., Parfrey, H., Rassl, D., Russell, A.-M., Saini, G., Renzoni, E. A., Duggan, A.-M., Hubbard, R., Wells, A. U., Lukey, P. T., Marshall, R. P., & Jenkins, R. G.
Affiliations: National Institute for Health and Care Research (GB)
GlaxoSmithKline (United Kingdom) (GB)
Nottingham University Hospitals NHS Trust (GB)
University of Nottingham (GB)
Royal Papworth Hospital (GB)
ORCID: Maher, Toby M
Porte, Joanne
Habgood, Anthony
Fahy, William A
Molyneaux, Philip L
Braybrooke, Rebecca
Parfrey, Helen
Rassl, Doris
Russell, Anne-Marie
Saini, Gauri
Renzoni, Elisabetta A
Hubbard, Richard
Wells, Athol U
Lukey, Pauline T
Marshall, Richard P
Venue: The Lancet Respiratory Medicine
Publisher: Elsevier BV
Published: 2017-12
Volume: 5
Issue: 12
Pages: 946-955
ISSN: 2213-2600 (ISSN-L)
DOI: 10.1016/s2213-2600(17)30430-7
OpenAlex: W2770778260
Cited by: 271
References: 34
Citations by year: 2026: 21 · 2025: 28 · 2024: 39 · 2023: 37 · 2022: 33 · 2021: 36 · 2020: 36 · 2019: 26 · 2018: 14 · 2017: 1
FWCI: 17.28
Type: journal article
Language: en
Open access: green
doi.org
Licence: other-oa
www.elsevier.com/tdm/userlicense/1.0/ (tdm)
Locations: The Lancet Respiratory Medicine (publishedVersion)
PubMed (publishedVersion)
Repository@Nottingham (University of Nottingham) (acceptedVersion, other-oa)
Repository@Nottingham (University of Nottingham) (submittedVersion, cc-by)
Nottingham ePrints (University of Nottingham) (submittedVersion, cc-by)
University of Birmingham Research Portal (University of Birmingham) (submittedVersion)
Spiral (Imperial College London) (submittedVersion, cc-by-nc-nd)
Funders: National Institute for Health and Care Research (PB-PG-0712-28073, CS-2013-13-017, CDRF-2012-03-053)
Versus Arthritis (20719)
Medical Research Council (MR/N005953/1)
Topics: Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Occupational and environmental lung diseases
Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Chronic Obstructive Pulmonary Disease (COPD) Research
Keywords: idiopathic pulmonary fibrosis, serum biomarkers, surfactant protein D, CA19-9, CA125, matrix metalloproteinase-7, disease progression prediction, mortality risk, epithelial damage, multiplex immunoassay, treatment-naive patients, lung histology, metaplastic epithelium, biomarker discovery
MeSH terms: Enzyme-Linked Immunosorbent Assay, Epithelium, Female, Humans, Longitudinal Studies, Lung, Male, Predictive Value of Tests, Prospective Studies, Biomarkers, Case-Control Studies, CA-125 Antigen, CA-19-9 Antigen, Disease Progression, Matrix Metalloproteinase 7, Pulmonary Surfactant-Associated Protein D, Idiopathic Pulmonary Fibrosis

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An epithelial biomarker signature for idiopathic pulmonary fibrosis: an analysis from the multicentre PROFILE cohort study

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