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

J Jaffar, S Unger, TJ Corte, M Keller, PJ Wolters… - Chest, 2014 - Elsevier
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 …