Pharmacological treatment of idiopathic pulmonary fibrosis and fibrosing interstitial lung diseases: current trends and future directions

Idiopathic pulmonary fibrosis (IPF) was considered untreatable until the development of therapeutic drugs and diagnostic technology that made it possible to slow the progression of IPF. In 2014, pirfenidone and nintedanib were approved simultaneously as therapeutic drugs for patients with IPF. These...

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Main Author: Won-Il Choi
Format: Article
Language:English
Published: Sungkyunkwan University School of Medi 2021-03-01
Series:Precision and Future Medicine
Subjects:
Online Access:http://www.pfmjournal.org/upload/pdf/pfm-2020-00205.pdf
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author Won-Il Choi
author_facet Won-Il Choi
author_sort Won-Il Choi
collection DOAJ
description Idiopathic pulmonary fibrosis (IPF) was considered untreatable until the development of therapeutic drugs and diagnostic technology that made it possible to slow the progression of IPF. In 2014, pirfenidone and nintedanib were approved simultaneously as therapeutic drugs for patients with IPF. These drugs have proven effective in reducing further progression of pulmonary fibrosis, acute exacerbation, and mortality and have consistent effects regardless of the severity of IPF. The indications of nintedanib and pirfenidone are gradually expanding for various other diseases that cause pulmonary fibrosis. Currently, IPF pathogenesis is associated with the type 2 alveolar epithelium and repeated or persistent damage to its cells. Such damage may induce an abnormal wound healing response, causing fibrosis rather than repair. Several promising drugs have been developed for reducing or reversing fibrosis, each with a different molecular target implicated in pulmonary fibrosis. Due to the heterogeneous mechanisms underlying pulmonary fibrosis, future disease management is likely to comprise combinations of therapies targeting a range of disease mechanisms.
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spelling doaj.art-df3b6868c4e541ea9dd7ecc2edd46b1f2022-12-21T17:13:24ZengSungkyunkwan University School of MediPrecision and Future Medicine2508-79402508-79592021-03-0151314010.23838/pfm.2020.0020591Pharmacological treatment of idiopathic pulmonary fibrosis and fibrosing interstitial lung diseases: current trends and future directionsWon-Il Choi0 Department of Internal Medicine, Myongji Hospital, Hanyang University College of Medicine, Goyang, KoreaIdiopathic pulmonary fibrosis (IPF) was considered untreatable until the development of therapeutic drugs and diagnostic technology that made it possible to slow the progression of IPF. In 2014, pirfenidone and nintedanib were approved simultaneously as therapeutic drugs for patients with IPF. These drugs have proven effective in reducing further progression of pulmonary fibrosis, acute exacerbation, and mortality and have consistent effects regardless of the severity of IPF. The indications of nintedanib and pirfenidone are gradually expanding for various other diseases that cause pulmonary fibrosis. Currently, IPF pathogenesis is associated with the type 2 alveolar epithelium and repeated or persistent damage to its cells. Such damage may induce an abnormal wound healing response, causing fibrosis rather than repair. Several promising drugs have been developed for reducing or reversing fibrosis, each with a different molecular target implicated in pulmonary fibrosis. Due to the heterogeneous mechanisms underlying pulmonary fibrosis, future disease management is likely to comprise combinations of therapies targeting a range of disease mechanisms.http://www.pfmjournal.org/upload/pdf/pfm-2020-00205.pdfidiopathic pulmonary fibrosisnintedanibpirfenidone
spellingShingle Won-Il Choi
Pharmacological treatment of idiopathic pulmonary fibrosis and fibrosing interstitial lung diseases: current trends and future directions
Precision and Future Medicine
idiopathic pulmonary fibrosis
nintedanib
pirfenidone
title Pharmacological treatment of idiopathic pulmonary fibrosis and fibrosing interstitial lung diseases: current trends and future directions
title_full Pharmacological treatment of idiopathic pulmonary fibrosis and fibrosing interstitial lung diseases: current trends and future directions
title_fullStr Pharmacological treatment of idiopathic pulmonary fibrosis and fibrosing interstitial lung diseases: current trends and future directions
title_full_unstemmed Pharmacological treatment of idiopathic pulmonary fibrosis and fibrosing interstitial lung diseases: current trends and future directions
title_short Pharmacological treatment of idiopathic pulmonary fibrosis and fibrosing interstitial lung diseases: current trends and future directions
title_sort pharmacological treatment of idiopathic pulmonary fibrosis and fibrosing interstitial lung diseases current trends and future directions
topic idiopathic pulmonary fibrosis
nintedanib
pirfenidone
url http://www.pfmjournal.org/upload/pdf/pfm-2020-00205.pdf
work_keys_str_mv AT wonilchoi pharmacologicaltreatmentofidiopathicpulmonaryfibrosisandfibrosinginterstitiallungdiseasescurrenttrendsandfuturedirections