Location(s): United States
Idiopathic pulmonary fibrosis is believed to be the sequela of abnormal healing of the lung in response to an injury and is characterized by lung injury, destruction of normal lung architecture, and abnormal deposition of collagens. A prominent feature of this disease is the organization of fibroblasts in specific regions of the lung termed fibroblast foci. Why the IPF fibroblasts organize into specific foci and why they secrete excessive quantities of collagen that cause fibrosis is unknown. One hypothesis is that the fibroblasts are intrinsically abnormal and that there abnormal phenotype drives the fibrotic process. To investigate this hypothesis, we have been using gene-profiling techniques to phenotype fibroblasts obtained from IPF patients with the objective of identifying and characterizing genes uniquely expressed in these cells that contribute to the fibrotic process.