Cellular Mechanisms in the Pathogenesis of Idiopathic Pulmonary Fibrosis
Sandra Cuerpo 1, Nuria Albacar 1, Mauricio Rojas 2
1 Servei de Pneumologia, Respiratory Institute, Hospital Clínic, IDIBAPS, Universitat de Barcelona, Barcelona, Spain; 2 Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, and The Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease, University of Pittsburgh Medical Center, Pittsburgh, PA, USA; McGowan Institute for Regenerative Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
*Correspondence: Mauricio Rojas, Email not available
Abstract
Current evidence suggests that the fibrotic response in idiopathic pulmonary fibrosis (IPF) arises due to accelerated aging of the lung after repeated injuries to the alveolar epithelium, which causes the production of inflammatory mediators that induce the recruitment and activation of lung fibroblasts. These cells are responsible for the secretion of excessive amounts of collagen fibers, destroying the normal lung architecture leading to decreased lung compliance, disrupted gas exchange, and, ultimately, respiratory failure. Different alterations of cellular function have been proposed to be related to the premature aging of pulmonary tissue in IPF, such as defects in DNA repair, mitochondrial dysfunction, telomeric shortening, loss of protein homeostasis, and cellular senescence. Improving our understanding of the pathophysiology of this disease is a crucial point to find new therapeutic targets. In the following article, we review recent data on the underlying mechanisms thought to be involved in the pathogenesis of IPF.
