The primary objective of this proposal is to provide a more precise characterization of the factors that predict pulmonary fibrosis (PF) development and progression in specific populations with the goal of defining groups that might benefit from participation in early PF screening studies. Idiopathic pulmonary fibrosis (IPF), the most common and severe form of pulmonary fibrosis (PF) has a mortality rate comparable to that of many end- stage malignancies Although IPF has historically been unresponsive to pharmacotherapy, recent studies have demonstrated that medical therapy can reduce the rate of decline in lung function, particularly when started early in the course of disease. Our recent findings demonstrated that early disease detection for PF is an achievable goal. In 1st degree relatives of patients with PF we have demonstrated ILA in 38% of those we have evaluated, and 33% of those with ILA were found to have signs of more advanced disease. These relatives have been referred for clinical evaluations, some of whom have begun on anti-fibrotic therapy for IPF/familial PF. While these findings demonstrate that a landmark shift from reacting - to preventing ? PF progression in close relatives is possible, it is not known the extent to which early PF can be detected in other unique populations at risk. Based on these findings we hypothesize that measurable characteristics can be identified in 1) smokers with and without COPD, 2) in patients with early stage lung cancer, and 3) in those with prior imaging abnormalities, that will help to distinguish those who already have PF (and those with the greatest risk to progress from early stages of PF) from those unlikely to develop this disease. The results of these studies will improve our understanding of early disease detection for PF, as well as setting the stage for trials aimed at the recruiting these specific populations for early institution of novel and existing medical therapies.
Idiopathic pulmonary fibrosis, a disorder characterized by lung scarring that has a prognosis worse than that of most cancers, can be slowed with medications targeting fibrosis but it is frequently identified in advanced stages. Our work suggests that early stages of pulmonary fibrosis can be detected in screening studies in first-degree relatives of patients with pulmonary fibrosis. This proposal is designed to provide a more precise characterization of the factors that predict pulmonary fibrosis development and progression in additional populations with the goal of defining groups that might benefit from participation in early screening studies.
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