Source: Annual Congress 2011 - Bronchoalveolar lavage and biomarkers in diffuse parenchymal lung disease
Disease area: Interstitial lung diseases

Abstract

Rationale: Idiopathic pulmonary fibrosis (IPF) is a progressive parenchymal lung disease of unknown aetiology and poor prognosis, characterized by progressive fibrosis of the alveolar interstitium. The ECM is a critical component in regulating cellular homeostasis and appropriate wound healing. The aim of our study was to determine the extracellular matrix protein profile of fibrotic lungs.
Methods: ECM gene expression and protein profile of lung of 12 patients with IPF, 5 chronic obstructive pulmonary disease (COPD) patients with emphysema and 7 subjects with pneumothorax (control group) were analysed by cDNA microarrays, rt-PCR and immunohistochemistry.
Results: Microarray analyze showed a total of 20 ECM proteins upregulated and 6 proteins downregulated in fibrotic lungs compared with normal lungs. Interstitial structural proteins like collagen I and III had a significant increase in IPF compared with normal lungs and emphysema. Migratory proteins (tenascin C and versican) were also significant augmented in fibrosis. These proteins were located in fibroblastic foci by immunohistochemistry. The main basal membrane protein, collagen IV, were downregulated in fibrotic lungs. There was a statistically significant increase of some metalloproteins in lungs of IPF patients compared with normal and emphysema lungs (MMP-1, MMP-7, MMP-9, MMP-12 and MMP-13).
Conclusion: ECM protein profile of fibrotic lungs show profibrogenic properties that could help disease progression and perpetuation of lung fibrosis.These findings should be considered in the IPF physiopathology for future antifibrotic therapeutic approaches.
Supported by: SOCAP, SEPAR, FUCAP, PS09/01757

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S. Estany, V. Vicens, R. Llatjós, R. Penín, I. Escobar, A. Xaubet, F. Manresa, J. Dorca, M. Molina-Molina (L‘Hospitalet de Llobregat, Barcelona, Spain). Extracellular matrix profile of lung in idiopathic pulmonary fibrosis. Eur Respir J 2011; 38: Suppl. 55, 4775

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