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Fibulin-1 predicts disease progression in patients with idiopathic pulmonary fibrosis

Fibulin-1 predicts disease progression in patients with idiopathic pulmonary fibrosis
Fibulin-1 predicts disease progression in patients with idiopathic pulmonary fibrosis
BACKGROUND The underlying mechanisms of idiopathic pulmonary fibrosis (IPF) are unknown. This progressive disease has high mortality rates and current models for prediction of mortality have limited value in identifying which patients will progress. We previously showed that the glycoprotein fibulin-1 is involved in enhanced proliferation and wound repair by mesenchymal cells, thus may contribute to lung fibrosis in IPF. METHODS Serum, lung tissue and lung function values were obtained from four independent locations (Sydney and Perth, Australia, San Francisco, USA and Modena, Italy). Patients with IPF were followed for a minimum of one year and progression was defined as a significant decline in lung function or death. Primary parenchymal lung fibroblasts of 15 patients with and without IPF were cultured under non-stimulatory conditions. Fibulin-1 levels in serum and secreted or deposited by fibroblasts were measured by western blot and in lung tissue by immunohistochemistry. RESULTS Serum fibulin-1 levels were increased in patients with IPF compared to subjects without lung disease (p=0.006). Furthermore, tissue fibulin-1 levels were increased in patients with IPF (p=0.02) and correlated negatively with lung function (r=-0.9, p<0.05). Primary parenchymal fibroblasts from patients with IPF produced more fibulin-1 than those from subjects without IPF (p<0.05). Finally, serum fibulin-1 levels at first blood draw predicted disease progression in IPF within 1 year (AUC 0.71, 95%CI 0.57 - 0.86, p=0.012). CONCLUSIONS Fibulin-1 is a novel potential biomarker for disease progression IPF and raise the possibility that it could be used as a target for the development of new treatments.
0012-3692
Jaffar, Jade
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Unger, Sofia
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Corte, Tamera J.
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Keller, Michael
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Wolters, Paul J.
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Richeldi, Luca
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Cerri, Stefania
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Prêle, Cecilia M.
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Hansbro, Philip M.
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Argraves, William Scott
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Oliver, Rema A.
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Oliver, Brian G.
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Black, Judith L.
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Burgess, Janette K.
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Jaffar, Jade
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Unger, Sofia
a2e36084-fc15-437a-a856-5f0436d2af6e
Corte, Tamera J.
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Keller, Michael
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Wolters, Paul J.
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Richeldi, Luca
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Cerri, Stefania
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Prêle, Cecilia M.
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Hansbro, Philip M.
f764d36e-4b05-4edd-bcaf-08526d79de25
Argraves, William Scott
ad7196d0-cad1-4fa4-be95-5baa2b5265e6
Oliver, Rema A.
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Oliver, Brian G.
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Black, Judith L.
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Burgess, Janette K.
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Jaffar, Jade, Unger, Sofia, Corte, Tamera J., Keller, Michael, Wolters, Paul J., Richeldi, Luca, Cerri, Stefania, Prêle, Cecilia M., Hansbro, Philip M., Argraves, William Scott, Oliver, Rema A., Oliver, Brian G., Black, Judith L. and Burgess, Janette K. (2014) Fibulin-1 predicts disease progression in patients with idiopathic pulmonary fibrosis. Chest. (doi:10.1378/chest.13-2688). (PMID:24832167)

Record type: Article

Abstract

BACKGROUND The underlying mechanisms of idiopathic pulmonary fibrosis (IPF) are unknown. This progressive disease has high mortality rates and current models for prediction of mortality have limited value in identifying which patients will progress. We previously showed that the glycoprotein fibulin-1 is involved in enhanced proliferation and wound repair by mesenchymal cells, thus may contribute to lung fibrosis in IPF. METHODS Serum, lung tissue and lung function values were obtained from four independent locations (Sydney and Perth, Australia, San Francisco, USA and Modena, Italy). Patients with IPF were followed for a minimum of one year and progression was defined as a significant decline in lung function or death. Primary parenchymal lung fibroblasts of 15 patients with and without IPF were cultured under non-stimulatory conditions. Fibulin-1 levels in serum and secreted or deposited by fibroblasts were measured by western blot and in lung tissue by immunohistochemistry. RESULTS Serum fibulin-1 levels were increased in patients with IPF compared to subjects without lung disease (p=0.006). Furthermore, tissue fibulin-1 levels were increased in patients with IPF (p=0.02) and correlated negatively with lung function (r=-0.9, p<0.05). Primary parenchymal fibroblasts from patients with IPF produced more fibulin-1 than those from subjects without IPF (p<0.05). Finally, serum fibulin-1 levels at first blood draw predicted disease progression in IPF within 1 year (AUC 0.71, 95%CI 0.57 - 0.86, p=0.012). CONCLUSIONS Fibulin-1 is a novel potential biomarker for disease progression IPF and raise the possibility that it could be used as a target for the development of new treatments.

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e-pub ahead of print date: 15 May 2014
Organisations: Clinical & Experimental Sciences

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Local EPrints ID: 369049
URI: https://eprints.soton.ac.uk/id/eprint/369049
ISSN: 0012-3692
PURE UUID: dd24efc7-a004-4ace-b184-2a0f77dd6ac5

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Date deposited: 18 Sep 2014 16:10
Last modified: 18 Jul 2017 01:40

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Contributors

Author: Jade Jaffar
Author: Sofia Unger
Author: Tamera J. Corte
Author: Michael Keller
Author: Paul J. Wolters
Author: Luca Richeldi
Author: Stefania Cerri
Author: Cecilia M. Prêle
Author: Philip M. Hansbro
Author: William Scott Argraves
Author: Rema A. Oliver
Author: Brian G. Oliver
Author: Judith L. Black
Author: Janette K. Burgess

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