Update on therapeutic management of idiopathic pulmonary fibrosis

Therapeutics and Clinical Risk Management
Argyris TzouvelekisPaolo Spagnolo

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive diffuse parenchymal lung disease of unknown origin, with a mortality rate exceeding that of many cancers. The diagnostic process is complex and relies on the clinician integrating clinical, laboratory, radiological, and histological data. In the last decade, major advances in our understanding of the pathogenesis of IPF have shifted the paradigm from a primarily inflammatory process evolving to fibrosis to a condition driven by aberrant wound healing following alveolar epithelial cell injury that results in scarring of the lung, architectural distortion, and irreversible loss of function. Improved understanding of disease pathogenesis has led to the identification of several therapeutic targets and the design of high-quality clinical trials evaluating novel compounds. However, the results of these studies have been mostly disappointing, probably due to the plethora of mediators, growth factors, and signaling pathways involved in the fibrotic process. Most recently, pirfenidone and nintedanib, two compounds with pleiotropic anti-fibrotic properties, have been proven effective in reducing functional decline and disease progression in IPF. This is a major breakthrough. ...Continue Reading

Citations

Aug 12, 2015·Virchows Archiv : an International Journal of Pathology·Elly De VlieghereOlivier De Wever
Aug 1, 2015·PloS One·Alejandro Guzmán-SilvaRoberto Berra-Romani
Nov 21, 2019·American Journal of Physiology. Lung Cellular and Molecular Physiology·Andrew RaskyNicholas W Lukacs
Dec 20, 2017·Oncotarget·Xiaohong LiZiqiang Luo
Aug 28, 2020·Journal of Comparative Effectiveness Research·Mitra CorralSheila R Reddy
Jun 6, 2018·Frontiers in Medicine·Argyrios TzouvelekisMarilyn K Glassberg
Sep 7, 2019·The Journal of Biological Chemistry·Junsuk KoMichael R Blackburn
Mar 1, 2018·Respiration; International Review of Thoracic Diseases·Eva BrunnemerMichael Kreuter
Feb 7, 2018·Frontiers in Medicine·Ourania PapaioannouArgyrios Tzouvelekis
Aug 15, 2017·Frontiers in Medicine·Jun TashiroMarilyn K Glassberg
Dec 15, 2017·Frontiers in Medicine·Argyrios TzouvelekisDemosthenes Bouros
Mar 27, 2021·Frontiers in Cell and Developmental Biology·Shengnan YangChen Wang

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Methods Mentioned

BETA
bronchoalveolar lavage

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TOMORROW
INSPIRE

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