• Paul J Wolters, Harold R Collard, and Kirk D Jones.
• Department of Medicine, School of Medicine, University of California, San Francisco, California 94143; email: paul.wolters@ucsf.edu , hal.collard@ucsf.edu.
• Annu Rev Pathol. 2014 Jan 1;9:157-79.

AbstractIdiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease associated with aging that is characterized by the histopathological pattern of usual interstitial pneumonia. Although an understanding of the pathogenesis of IPF is incomplete, recent advances delineating specific clinical and pathologic features of IPF have led to better definition of the molecular pathways that are pathologically activated in the disease. In this review we highlight several of these advances, with a focus on genetic predisposition to IPF and how genetic changes, which occur primarily in epithelial cells, lead to activation of profibrotic pathways in epithelial cells. We then discuss the pathologic changes within IPF fibroblasts and the extracellular matrix, and we conclude with a summary of how these profibrotic pathways may be interrelated.

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