Idiopathic pulmonary fibrosis: Disease mechanisms and drug development — spagnolo 2020

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive condition with no known cause that causes the lung parenchyma to scar incessantly, lowering quality of life and hastening death. IPF is an age-related condition, and as the world's population ages, its economic toll is anticipated to rise over time. The disease's pathophysiology is currently thought to include repeated microinjuries to an alveolar epithelium that is genetically predisposed, followed by an abnormal reparative response that is marked by excessive collagen deposition. Based on their capacity to reduce disease development and functional deterioration, pirfenidone and nintedanib are licensed for the treatment of IPF. Despite this, they do not provide a cure and have tolerability problems. In this review, we critically examine the potential benefits of new therapeutic targets being discovered as a result of cutting-edge research on disease pathophysiology. There are more and more therapy options available for IPF. However, a combination of treatment approaches with various mechanisms of action may be necessary to target the large number of profibrotic cytokines and growth factors implicated in disease development.

Spagnolo P, Kropski JA, Jones MG, Lee JS, Rossi G, Karampitsakos T, Maher TM, Tzouvelekis A, Ryerson CJ. Idiopathic pulmonary fibrosis: Disease mechanisms and drug development. Pharmacol Ther. 2021 Jun;222:107798. doi: 10.1016/j.pharmthera.2020.107798. Epub 2020 Dec 24. PMID: 33359599; PMCID: PMC8142468.