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Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis is a prototype of chronic, progressive, and fibrotic lung disease. Healthy tissue is replaced by altered extracellular matrix and alveolar architecture is destroyed, which leads to decreased lung compliance, disrupted gas exchange, and ultimately respiratory failure and death. In less than a decade, understanding of the pathogenesis and management of this disease has been transformed, and two disease-modifying therapies have been approved, worldwide. In this Seminar, we summarise the presentation, pathophysiology, diagnosis, and treatment options available for patients with idiopathic pulmonary fibrosis. This disease has improved understanding of the mechanisms of lung fibrosis, and offers hope that similar approaches will transform the management of patients with other progressive fibrotic lung diseases.
0140-6736
Richeldi, Luca
47177d9c-731a-49a1-9cc6-4ac8f6bbbf26
Collard, Harold R.
8eebf6ae-6572-4112-af1c-45b5914c40ca
Jones, Mark G.
a6fd492e-058e-4e84-a486-34c6035429c1
Richeldi, Luca
47177d9c-731a-49a1-9cc6-4ac8f6bbbf26
Collard, Harold R.
8eebf6ae-6572-4112-af1c-45b5914c40ca
Jones, Mark G.
a6fd492e-058e-4e84-a486-34c6035429c1

Richeldi, Luca, Collard, Harold R. and Jones, Mark G. (2017) Idiopathic pulmonary fibrosis. The Lancet. (doi:10.1016/S0140-6736(17)30866-8).

Record type: Review

Abstract

Idiopathic pulmonary fibrosis is a prototype of chronic, progressive, and fibrotic lung disease. Healthy tissue is replaced by altered extracellular matrix and alveolar architecture is destroyed, which leads to decreased lung compliance, disrupted gas exchange, and ultimately respiratory failure and death. In less than a decade, understanding of the pathogenesis and management of this disease has been transformed, and two disease-modifying therapies have been approved, worldwide. In this Seminar, we summarise the presentation, pathophysiology, diagnosis, and treatment options available for patients with idiopathic pulmonary fibrosis. This disease has improved understanding of the mechanisms of lung fibrosis, and offers hope that similar approaches will transform the management of patients with other progressive fibrotic lung diseases.

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Idiopathic Pulmonary Fibrosis Lancet Seminar 2017 - Accepted Manuscript
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Accepted/In Press date: 3 March 2017
e-pub ahead of print date: 30 March 2017
Organisations: Clinical & Experimental Sciences

Identifiers

Local EPrints ID: 407758
URI: https://eprints.soton.ac.uk/id/eprint/407758
ISSN: 0140-6736
PURE UUID: da14be8e-9a60-4f01-8dd7-c8620ea29ad1

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Date deposited: 25 Apr 2017 01:08
Last modified: 10 Dec 2019 06:06

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