Imperial College London
Alternate

Professor Toby Maher

Faculty of Medicine, National Heart & Lung Institute

Professor of Interstitial Lung Disease
 
 
 
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Contact

 

+44 (0)20 7594 2151t.maher

 
 
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Assistant

 

Ms Georgina Moss +44 (0)20 7594 2151

 
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Location

 

364Sir Alexander Fleming BuildingSouth Kensington Campus

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Summary

 

Publications

Citation

BibTex format

@article{Spagnolo:2021:10.1016/j.pharmthera.2020.107798,
author = {Spagnolo, P and Kropski, JA and Jones, MG and Lee, JS and Rossi, G and Karampitsakos, T and Maher, TM and Tzouvelekis, A and Ryerson, CJ},
doi = {10.1016/j.pharmthera.2020.107798},
journal = {Pharmacology and Therapeutics},
pages = {107798--107798},
title = {Idiopathic pulmonary fibrosis: Disease mechanisms and drug development},
url = {http://dx.doi.org/10.1016/j.pharmthera.2020.107798},
volume = {222},
year = {2021}
}
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RIS format (EndNote, RefMan)

TY  - JOUR
AB  - 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.
AU  - Spagnolo,P
AU  - Kropski,JA
AU  - Jones,MG
AU  - Lee,JS
AU  - Rossi,G
AU  - Karampitsakos,T
AU  - Maher,TM
AU  - Tzouvelekis,A
AU  - Ryerson,CJ
DO  - 10.1016/j.pharmthera.2020.107798
EP  - 107798
PY  - 2021///
SN  - 0163-7258
SP  - 107798
TI  - Idiopathic pulmonary fibrosis: Disease mechanisms and drug development
T2  - Pharmacology and Therapeutics
UR  - http://dx.doi.org/10.1016/j.pharmthera.2020.107798
UR  - https://www.ncbi.nlm.nih.gov/pubmed/33359599
UR  - https://www.sciencedirect.com/science/article/pii/S0163725820303296?via%3Dihub
UR  - http://hdl.handle.net/10044/1/86282
VL  - 222
ER  -
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