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Recent advances and future needs in interstitial lung diseases

Recent advances and future needs in interstitial lung diseases
Recent advances and future needs in interstitial lung diseases

Interstitial lung diseases (ILDs) are a diverse range of conditions affecting the lung interstitium. The prototypic ILD, idiopathic pulmonary fibrosis (IPF), is a chronic progressive fibrotic lung disease with a median survival of only 3 years from the time of diagnosis. Recently significant progress has been made in both our understanding of the pathogenesis and of the therapeutic targeting of IPF. This culminated in the worldwide approval of the first antifibrotic therapies nintedanib and pirfenidone. While an important first step, patients continue to progress and better therapies are urgently required. The aim of this article is to highlight some of the recent advances that have been made in our understanding of genetics, disease classification, clinical trial design, and novel antifibrotic therapy in IPF. It discusses future priorities if we are to continue to increase the length and quality of life of patients with IPF, and considers possible approaches to translate the progress made in IPF to other progressive fibrotic lung diseases where our understanding remains limited.

Clinical Trials as Topic, Forecasting, Humans, Idiopathic Pulmonary Fibrosis/diagnosis, Indoles/therapeutic use, Lung Diseases, Interstitial/diagnosis, Pyridones/therapeutic use, Quality of Life
1069-3424
477-484
Jones, Mark G.
a6fd492e-058e-4e84-a486-34c6035429c1
Richeldi, Luca
47177d9c-731a-49a1-9cc6-4ac8f6bbbf26
Jones, Mark G.
a6fd492e-058e-4e84-a486-34c6035429c1
Richeldi, Luca
47177d9c-731a-49a1-9cc6-4ac8f6bbbf26

Jones, Mark G. and Richeldi, Luca (2016) Recent advances and future needs in interstitial lung diseases. Seminars in Respiratory and Critical Care Medicine, 37 (3), 477-484. (doi:10.1055/s-0036-1580688).

Record type: Review

Abstract

Interstitial lung diseases (ILDs) are a diverse range of conditions affecting the lung interstitium. The prototypic ILD, idiopathic pulmonary fibrosis (IPF), is a chronic progressive fibrotic lung disease with a median survival of only 3 years from the time of diagnosis. Recently significant progress has been made in both our understanding of the pathogenesis and of the therapeutic targeting of IPF. This culminated in the worldwide approval of the first antifibrotic therapies nintedanib and pirfenidone. While an important first step, patients continue to progress and better therapies are urgently required. The aim of this article is to highlight some of the recent advances that have been made in our understanding of genetics, disease classification, clinical trial design, and novel antifibrotic therapy in IPF. It discusses future priorities if we are to continue to increase the length and quality of life of patients with IPF, and considers possible approaches to translate the progress made in IPF to other progressive fibrotic lung diseases where our understanding remains limited.

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More information

Published date: June 2016
Keywords: Clinical Trials as Topic, Forecasting, Humans, Idiopathic Pulmonary Fibrosis/diagnosis, Indoles/therapeutic use, Lung Diseases, Interstitial/diagnosis, Pyridones/therapeutic use, Quality of Life

Identifiers

Local EPrints ID: 442206
URI: http://eprints.soton.ac.uk/id/eprint/442206
ISSN: 1069-3424
PURE UUID: 00d78acf-0d83-49aa-b1ee-764ee357cada
ORCID for Mark G. Jones: ORCID iD orcid.org/0000-0001-6308-6014

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Date deposited: 08 Jul 2020 16:39
Last modified: 17 Mar 2024 03:11

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Contributors

Author: Mark G. Jones ORCID iD
Author: Luca Richeldi

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