Pathogenesis and New Diagnosis Guideline of Idiopathic Pulmonary Fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal fibrotic lung disease in many patients. In spite of extensive research for many decades, the exact pathogenesis of IPF is unknown. At recent, the role of alveolar epithelial cells has been focused in the initiation of IPF in terms of epithelial-mesenchymal transition, dysregulated Wnt signaling, and activation of transforming growth factor-β (TGF-β). The resulting excess collagen deposition and destruction of lung architecture by myofibroblasts and fibroblastic foci leads to the development of IPF. IPF can be diagnosed by typical high resolution chest tomogram (HRCT) or by multidisciplinary discussion based on the new guideline published on 2010. (Korean J Med 2013;84:481-488)