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Idiopathic pulmonary fibrosis: recent advances on pharmacological therapy

Idiopathic pulmonary fibrosis: recent advances on pharmacological therapy
Idiopathic pulmonary fibrosis: recent advances on pharmacological therapy
Idiopathic pulmonary fibrosis (IPF) is the most common and lethal of the idiopathic interstitial pneumonias with an estimated 5-year survival of approximately 20%. In the last two decades our understanding of disease pathogenesis has substantially evolved and novel compounds have been developed consequent to the increasing knowledge of the mechanisms underlying disease pathobiology. The disease appears to be driven – following chronic injury – by abnormal/dysfunctional alveolar epithelial cells that promote fibroblast recruitment and proliferation, resulting in scarring of the lung and irreversible loss of function. With very few exceptions, clinical trials evaluating novel potential therapies have provided disappointing results. More recently, pirfenidone and nintedanib, two compounds with pleiotropic mechanisms of action, have proven effective in slowing functional decline and disease progression in IPF patients with mild to moderate functional impairment, highlighting the importance of timely diagnosis and administration of treatment in early stages of disease. However, due to the complexity and uncertainties intrinsic to IPF, it is essential that each therapeutic strategy be tailored to the individual patient, after evaluation of potential benefits and risks. This article provides an overview of the most recent clinical trials in IPF and discusses how their results are going to change the clinical and clinical research landscape in IPF. A number of agents with high potential are currently being tested and many more are ready for clinical trials. Their completion is critical for achieving the ultimate goal of curing patients with IPF.
clinical trials, idiopathic pulmonary fibrosis, nintedanib, pirfenidone, therapy, treatment
0163-7258
1-10
Spagnolo, P.
dfc6600e-bbca-4678-abc4-6feb243d4fbb
Maher, T.M.
c6f3810d-d7bb-44d2-a58f-86300ad5aa2d
Richeldi, L.
47177d9c-731a-49a1-9cc6-4ac8f6bbbf26
Spagnolo, P.
dfc6600e-bbca-4678-abc4-6feb243d4fbb
Maher, T.M.
c6f3810d-d7bb-44d2-a58f-86300ad5aa2d
Richeldi, L.
47177d9c-731a-49a1-9cc6-4ac8f6bbbf26

Spagnolo, P., Maher, T.M. and Richeldi, L. (2015) Idiopathic pulmonary fibrosis: recent advances on pharmacological therapy. Pharmacology & Therapeutics, 1-10. (doi:10.1016/j.pharmthera.2015.04.005). (PMID:25946646)

Record type: Article

Abstract

Idiopathic pulmonary fibrosis (IPF) is the most common and lethal of the idiopathic interstitial pneumonias with an estimated 5-year survival of approximately 20%. In the last two decades our understanding of disease pathogenesis has substantially evolved and novel compounds have been developed consequent to the increasing knowledge of the mechanisms underlying disease pathobiology. The disease appears to be driven – following chronic injury – by abnormal/dysfunctional alveolar epithelial cells that promote fibroblast recruitment and proliferation, resulting in scarring of the lung and irreversible loss of function. With very few exceptions, clinical trials evaluating novel potential therapies have provided disappointing results. More recently, pirfenidone and nintedanib, two compounds with pleiotropic mechanisms of action, have proven effective in slowing functional decline and disease progression in IPF patients with mild to moderate functional impairment, highlighting the importance of timely diagnosis and administration of treatment in early stages of disease. However, due to the complexity and uncertainties intrinsic to IPF, it is essential that each therapeutic strategy be tailored to the individual patient, after evaluation of potential benefits and risks. This article provides an overview of the most recent clinical trials in IPF and discusses how their results are going to change the clinical and clinical research landscape in IPF. A number of agents with high potential are currently being tested and many more are ready for clinical trials. Their completion is critical for achieving the ultimate goal of curing patients with IPF.

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More information

e-pub ahead of print date: 3 May 2015
Keywords: clinical trials, idiopathic pulmonary fibrosis, nintedanib, pirfenidone, therapy, treatment
Organisations: Clinical & Experimental Sciences

Identifiers

Local EPrints ID: 376914
URI: https://eprints.soton.ac.uk/id/eprint/376914
ISSN: 0163-7258
PURE UUID: ab6c88ab-1711-4baf-b43b-c37e69908c3c

Catalogue record

Date deposited: 13 May 2015 09:24
Last modified: 17 Jul 2017 21:05

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