Journal article
Fibulin-1 predicts disease progression in patients with idiopathic pulmonary fibrosis
Chest, Vol.146(4), pp.1055-1063
2014
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 and, thus, may contribute to lung fibrosis in IPF.
METHODS
Serum, lung tissue, and lung function values were obtained from four independent locations (Sydney, NSW, and Perth, WA, Australia; San Francisco, CA; and Modena, Italy). Patients with IPF were followed for a minimum of 1 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 nonstimulatory 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 with subjects without lung disease (P = .006). Furthermore, tissue fibulin-1 levels were increased in patients with IPF (P = .02) and correlated negatively with lung function (r = −0.9, P < .05). Primary parenchymal fibroblasts from patients with IPF produced more fibulin-1 than those from subjects without IPF (P < .05). Finally, serum fibulin-1 levels at first blood draw predicted disease progression in IPF within 1 year (area under the curve, 0.71; 95% CI, 0.57-0.86; P = .012).
CONCLUSIONS
Fibulin-1 is a novel potential biomarker for disease progression in IPF and raises the possibility that it could be used as a target for the development of new treatments.
Details
- Title
- Fibulin-1 predicts disease progression in patients with idiopathic pulmonary fibrosis
- Authors/Creators
- J. Jaffar (Author/Creator)S. Unger (Author/Creator)T.J. Corte (Author/Creator)M. Keller (Author/Creator)P.J. Wolters (Author/Creator)L. Richeldi (Author/Creator)S. Cerri (Author/Creator)C.M. Prêle (Author/Creator)P.M. Hansbro (Author/Creator)W.S. Argraves (Author/Creator)R.A. Oliver (Author/Creator)B.G. Oliver (Author/Creator)J.L. Black (Author/Creator)J.K. Burgess (Author/Creator)
- Publication Details
- Chest, Vol.146(4), pp.1055-1063
- Publisher
- Elsevier
- Identifiers
- 991005541490607891
- Copyright
- © 2014 The American College of Chest Physicians.
- Murdoch Affiliation
- Murdoch University
- Language
- English
- Resource Type
- Journal article
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- Collaboration types
- Domestic collaboration
- International collaboration
- Citation topics
- 1 Clinical & Life Sciences
- 1.208 Vasculitis & Autoimmune Disorders
- 1.208.1262 Idiopathic Pulmonary Fibrosis
- Web Of Science research areas
- Critical Care Medicine
- Respiratory System
- ESI research areas
- Clinical Medicine