Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease that commonly results in respiratory failure and death with a median survival of three years after diagnosis. Recent studies demonstrate traditional therapy consisting of corticosteroids and cytotoxic agents to be ineffective in improving survival. There is currently no effective treatment for this disorder. Recent advances in basic research on the pathogenesis of IPF and the development of novel therapeutic agents have increased the need for a cooperative clinical research network in evaluating new management strategies. An established group of clinical investigators (Mayo Interstitial Lung Disease Group) with a longstanding interest and track record in IPF proposes the development of a clinical center at Mayo Clinic to participate in the NIH-funded IPF Clinical Research Network for conducting collaborative therapeutic trials. These clinical investigators and their research staff of the Pulmonary Clinical Research Center have considerable experience in recruitment and retention of subjects for clinical trials in IPF and other lung diseases. This clinical center proposes two treatment studies for consideration by the network. The first study will examine the effects of combined imatinib and pentoxifylline regimen compared to placebo in the treatment of IPF. The second project will study the effects of combined colchicines and N-acetylcysteine therapy compared to placebo. To conduct these and other IPF Clinical Research Network studies, this clinical center will draw from 200-250 new patients with IPF seen each year at Mayo Clinic Rochester. The Mayo Interstitial Lung Disease Clinic registry and computerized databases will allow identification and participation of other subjects with IPF previously seen at this clinical center. This clinical center will also have access to other potential participants seen at Mayo Clinic Jacksonville (FL) and Mayo Clinic Scottsdale (AZ).
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