Prospective Evaluation of Biomarker Profiles in Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal, fibrotic disorder of the lung. The estimated prevalence is 30-80/100,000 in the United States with incidence estimates clearly rising. A major challenge in the care of patients with IPF is determining prognosis. The natural history of IPF is usually one of inexorable decline in lung function, ultimately resulting in death from respiratory failure. However, longitudinal physiologic decline in IPF is heterogeneous and difficult to predict in individual patients. While some patients with IPF may remain stable for years, in others the disease may progress rapidly over a relatively short time. We hypothesize that peripheral blood biomarkers based on extracellular matrix and matrix-modifying molecules will improve prognostication in patients with IPF.