Idiopathic pulmonary fibrosis: Disease mechanisms and drug development.
Disease mechanisms
Genomics
Idiopathic pulmonary fibrosis
Pathogenesis
Single-cell biology
Stem cells
Therapeutic targets
Treatment
Journal
Pharmacology & therapeutics
ISSN: 1879-016X
Titre abrégé: Pharmacol Ther
Pays: England
ID NLM: 7905840
Informations de publication
Date de publication:
06 2021
06 2021
Historique:
received:
11
11
2020
accepted:
14
12
2020
pubmed:
29
12
2020
medline:
15
1
2022
entrez:
28
12
2020
Statut:
ppublish
Résumé
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive disease of unknown cause characterized by relentless scarring of the lung parenchyma leading to reduced quality of life and earlier mortality. IPF is an age-related disorder, and with the population aging worldwide, the economic burden of IPF is expected to steadily increase in the future. The mechanisms of fibrosis in IPF remain elusive, with favored concepts of disease pathogenesis involving recurrent microinjuries to a genetically predisposed alveolar epithelium, followed by an aberrant reparative response characterized by excessive collagen deposition. Pirfenidone and nintedanib are approved for treatment of IPF based on their ability to slow functional decline and disease progression; however, they do not offer a cure and are associated with tolerability issues. In this review, we critically discuss how cutting-edge research in disease pathogenesis may translate into identification of new therapeutic targets, thus facilitate drug discovery. There is a growing portfolio of treatment options for IPF. However, targeting the multitude of profibrotic cytokines and growth factors involved in disease pathogenesis may require a combination of therapeutic strategies with different mechanisms of action.
Identifiants
pubmed: 33359599
pii: S0163-7258(20)30329-6
doi: 10.1016/j.pharmthera.2020.107798
pmc: PMC8142468
mid: NIHMS1703203
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
107798Subventions
Organisme : NHLBI NIH HHS
ID : K08 HL130595
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL145372
Pays : United States
Organisme : Doris Duke Charitable Foundation
ID : 2018099
Pays : United States
Organisme : Department of Health
ID : CS-2013-13-017
Pays : United Kingdom
Organisme : NHLBI NIH HHS
ID : R01 HL153246
Pays : United States
Informations de copyright
Copyright © 2020 Elsevier Inc. All rights reserved.
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