Idiopathic Pulmonary Fibrosis is a chronic, lethal lung disease without etiology. Currently, there is no medical therapy except lung transplantation which is more effective than any medical therapy available. Previous studies suggest Biological Markers (Biomarkers), protein or genes in human bodies may serve as predictors of diseases severity and mortality. The reported predictors of poor prognosis in the peripheral blood of patients with IPF include serum CCL-18, serum surfactant protein-A, and circulating fibrocytes. In our previous work, we discovered peripheral blood signatures in the peripheral blood of IPF patients that included MMP1 and MMP7 and distinguished IPF from controls and from COPD and Sarcoidosis. More recently we found an outcome predictive signature that included MMP7, IL8, S100A12 among others. The overall objective of this proposal is to transform the clinical management of patients with IPF by identifying and validating peripheral protein biomarkers in the accessible peripheral blood that are highly specific and reproducible, and relevant to disease mechanisms.

Public Health Relevance

IPF is a chronic, progressive and lethal lung disease. Currently there are no validated and accessible biomarkers for the disease. Identifying peripheral blood protein markers that are specific to the disease and are predictive of outcome will allow better patient management, improve access to transplant and facilitate clinical research and drug discovery and validation.

National Institute of Health (NIH)
Research Scientist Development Award - Research & Training (K01)
Project #
Application #
Study Section
Program Officer
Colombini-Hatch, Sandra
Project Start
Project End
Budget Start
Budget End
Support Year
Fiscal Year
Total Cost
Indirect Cost
University of Pittsburgh
Internal Medicine/Medicine
Schools of Medicine
United States
Zip Code
Vuga, Louis J; Tedrow, John R; Pandit, Kusum V et al. (2014) C-X-C motif chemokine 13 (CXCL13) is a prognostic biomarker of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 189:966-74
Leikauf, George D; Concel, Vincent J; Bein, Kiflai et al. (2013) Functional genomic assessment of phosgene-induced acute lung injury in mice. Am J Respir Cell Mol Biol 49:368-83