Idiopathic Pulmonary Fibrosis (IPF) is a chronic progressive lung disease with no proven therapy. The outcome in most patients includes gradual progression of restrictive lung disease to respiratory failure in half the patients within a period of 3-5 years. Estimates for prevalence suggest that there are 100,000 patients in the US with IPF, and it is the commonest cause of death among patients awaiting lung transplant for end-stage lung disease. Many available pharmaceutical agents have actions which would predict improved outcomes in IPF but have not been tested in patients. It is crucial that well-designed, multi-center, randomized, placebo-controlled trials be conducted promptly to examine whether an effective therapy for IPF can be identified. We plan to determine the effects of treatment of IPF with everolimus in a multi-center, randomized, placebo-controlled clinical trial which will enroll 240 IPF patients. In a subsequent trial of 360 IPF patients, we will determine the effects of treatment of IPF with prednisone or pirfenidone, or both, in a multi-center, randomized, placebo-controlled clinical trial. Finally, we will participate fully as a clinical center in all of the multi-center, randomized, placebo-controlled trials as directed by the Steering Committee of the IPF Clinical Research Network.

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Cooperative Clinical Research--Cooperative Agreements (U10)
Project #
5U10HL080370-05
Application #
7615152
Study Section
Special Emphasis Panel (ZHL1-CSR-M (F1))
Program Officer
Reynolds, Herbert Y
Project Start
2005-05-01
Project End
2012-04-30
Budget Start
2009-05-01
Budget End
2012-04-30
Support Year
5
Fiscal Year
2009
Total Cost
$204,767
Indirect Cost
Name
Vanderbilt University Medical Center
Department
Internal Medicine/Medicine
Type
Schools of Medicine
DUNS #
004413456
City
Nashville
State
TN
Country
United States
Zip Code
37212
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Swigris, Jeffrey J; Streiner, David L; Brown, Kevin K et al. (2014) Assessing exertional dyspnea in patients with idiopathic pulmonary fibrosis. Respir Med 108:181-8
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Han, MeiLan K; Bach, David S; Hagan, Peter G et al. (2013) Sildenafil preserves exercise capacity in patients with idiopathic pulmonary fibrosis and right-sided ventricular dysfunction. Chest 143:1699-1708
Idiopathic Pulmonary Fibrosis Clinical Research Network; Raghu, Ganesh; Anstrom, Kevin J et al. (2012) Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med 366:1968-77
Noth, Imre; Anstrom, Kevin J; Calvert, Sara Bristol et al. (2012) A placebo-controlled randomized trial of warfarin in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 186:88-95
Swigris, Jeffrey J; Han, Meilan; Vij, Rekha et al. (2012) The UCSD shortness of breath questionnaire has longitudinal construct validity in idiopathic pulmonary fibrosis. Respir Med 106:1447-55
(2010) The IPFnet Strategy: Creating a comprehensive approach in the treatment of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 181:527-8

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