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The matricellular protein Fibulin-1 is a better marker of disease progression than periostin, Tenascin-C and fibronectin in patients with idiopathic pulmonary fibrosis

Jaffar, J., Van Ly, D., Munk, L., Corte, T., Cerri, S., Richeldi, L., Wolters, P., Prêle, C., Oliver, R., Oliver, B., Black, J. and Burgess, J. (2014) The matricellular protein Fibulin-1 is a better marker of disease progression than periostin, Tenascin-C and fibronectin in patients with idiopathic pulmonary fibrosis. Respirology, 19 . pp. 32-34.

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Prediction of disease progression in idiopathic pulmonary fibrosis (IPF) remains challenging in the clinical setting. We have previously shown that the matricellular protein fibulin-1 enhances proliferation of mesenchymal cells. Increased levels of extracellular matrix (ECM) proteins may be a consequence of activated fibroblasts that produce excessive ECM proteins in the context of fibrotic disease.

The aim of this study was to compare the utility of the ECM proteins fibulin-1, periostin, tenascin-C and fibronectin as biomarkers of disease progression in patients with IPF.

Primary parenchymal fibroblasts derived from 8 patients with IPF and 7 subjects without lung disease were assessed for levels of cell-secreted ECM protein production. Serum and cell-secreted fibroblast proteins were measured by western blot (fibulin-1) and sandwich ELISA (periostin, tenascin-C, fibronectin) in 72 patients with IPF and 17 subjects without lung disease. Levels of the proteins were measured in the distal lung parenchyma of 20 patients with IPF and 5 subjects without lung disease using immunohistochemistry. Disease progression in patients with IPF was defined as a significant reduction in lung function or death within the first year of blood draw.

Fibulin-1 was the only of the 4 ECM proteins to accurately predict disease progression in patients with IPF (AUC 0.71, 95%CI 0.6 to 0.9, p = 0.01). Levels of fibulin-1 and periostin were increased in the serum and tissue of patients with IPF (p < 0.05) but only levels of fibulin-1 inversely correlated with lung function (r = −0.9, p < 0.05). In addition, only cell-secreted fibulin-1 production was increased in IPF fibroblasts compared to subjects without lung disease (p < 0.01).

The dysregulated levels of secreted ECM proteins can be reflected in the serum and act as biomarkers of activated fibroblasts and disease progression. Increased cell-secreted fibulin-1 production is an intrinsic quality of fibroblasts from patients with IPF and dysregulation was not seen in periostin or tenascin-C levels.

Item Type: Journal Article
Publisher: Wiley-Blackwell
Copyright: © 2014 The Authors/Asian Pacific Society of Respirology
Other Information: Ann Woolcock Young Investigator Award - Oral Presentations of the Thoracic Society of Australia & New Zealand and the Australian & New Zealand Society of Respiratory Science 2014 Annual Scientific Meetings, 4‐9 April 2014, Adelaide Convention Centre, Adelaide, SA
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