Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities.
File(s)
Author(s)
Type
Journal Article
Abstract
Within the spectrum of fibrosing interstitial lung diseases (ILDs) is a subset of patients who have inexorable progression of pulmonary fibrosis despite treatment, which is known as the progressive fibrotic phenotype. Although the concept of progressive fibrosing ILD has been applied largely to patients with idiopathic pulmonary fibrosis (IPF), there is now an increasing focus on irreversible progressive fibrosis in a proportion of patients with a range of underlying ILD diagnoses. Evidence has emerged to support a possible role for antifibrotic therapy in these patients. In this Position Paper, we discuss the importance of retaining diagnostic scrutiny within the multidisciplinary team and suggest a multidomain definition for progressive fibrosis. We consider the potential role of antifibrotic drugs as second-line therapy in the treatment algorithm for patients with progressive non-IPF ILD. We highlight risk factors that might predispose individuals to developing progressive fibrosis. Finally, we discuss key uncertainties and future directions for research and clinical practice.
Date Issued
2020-09
Date Acceptance
2020-07-28
Citation
The Lancet Respiratory Medicine, 2020, 8 (9), pp.925-934
ISSN
2213-2600
Publisher
Elsevier
Start Page
925
End Page
934
Journal / Book Title
The Lancet Respiratory Medicine
Volume
8
Issue
9
Copyright Statement
2020 © Elsevier Ltd. All rights reserved. This manuscript is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International Licence http://creativecommons.org/licenses/by-nc-nd/4.0/
Sponsor
The Jon Moulton Charity Trust
Action for Pulmonary Fibrosis
Identifier
https://www.ncbi.nlm.nih.gov/pubmed/32890499
PII: S2213-2600(20)30355-6
Grant Number
n/a
n/a
Subjects
Erice ILD working group
1103 Clinical Sciences
1117 Public Health and Health Services
1199 Other Medical and Health Sciences
Publication Status
Published
Coverage Spatial
England
Date Publish Online
2020-09-02