Work

Pathogenesis of Idiopathic Pulmonary Fibrosis

Year: 2013

Type: review

Abstract: Idiopathic 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.

Source: Annual Review of Pathology Mechanisms of Disease

DOI: 10.1146/annurev-pathol-012513-104706

Authors: Paul J. Wolters, Harold R. Collard, Kirk D. Jones

Institution: University of California, San Francisco

Language: English

Topic: Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis

Subfield: Pulmonary and Respiratory Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: bronze

Funders: National Institutes of Health, University of California, San Francisco, National Heart, Lung, and Blood Institute