Moisés Selman, National Institute of Respiratory Diseases Ismael Cosío Villegas, Mexico City, Mexico
Annie Pardo, Faculty of Sciences, Universidad Nacional Autónoma de México, Mexico City, Mexico
Idiopathic pulmonary fibrosis is a chronic, progressive, and usually lethal lung disease of unknown origin with a median survival time of 3-5 years after diagnosis. Although the pathogenic mechanisms and the sequence of the events leading to idiopathic pulmonary fibrosis have not been completely elucidated, a growing body of evidence indicates that the disease is caused by persistent epithelial injury and activation in genetically susceptible older individuals, with a signature of epigenetic changes that provokes the expansion of the fibroblast population and its differentiation to myofibroblasts, which secrete exaggerated amounts of extracellular matrix with the subsequent architectural remodeling and lung destruction. In this review, we discuss the clinical diagnosis and behavior of idiopathic pulmonary fibrosis and highlight the role of the genetic architecture, epigenetic modifications, and aging in the pathogenesis of the disease. Finally, we discuss the current therapeutic approaches, including the use of nintedanib and pirfenidone according to recently completed phase III idiopathic pulmonary fibrosis clinical trials.
Keywords: Aging. Epigenetic. Genome-wide association study. IPF. Pulmonary fibrosis.
