Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Editorial
. 2019 Oct 1;28(153):190109.
doi: 10.1183/16000617.0109-2019. Print 2019 Sep 30.

Treatment of progressive fibrosing interstitial lung diseases: a milestone in the management of interstitial lung diseases

Vincent Cottin  1
Affiliations
Editorial

Treatment of progressive fibrosing interstitial lung diseases: a milestone in the management of interstitial lung diseases

Vincent Cottin. Eur Respir Rev. .

Abstract

Fibrosing ILDs can develop a progressive phenotype and are described under the terminology PF-ILDs. Due to commonalities with IPF, the potential efficacy and tolerability of antifibrotic drugs pirfenidone and nintedanib are currently evaluated in PF-ILDs. http://bit.ly/2koL0A4

PubMed Disclaimer

Conflict of interest statement

Conflict of interest: V. Cottin reports personal fees and non-financial support from Actelion, grants, personal fees and non-financial support from Boehringer Ingelheim and Roche, and personal fees from Bayer/MSD, Gilead, Novartis, Sanofi, Promedior, Celgene, Galapagos and Galecto, outside the submitted work.

Figures

FIGURE 1
FIGURE 1
Schematic representation of types of interstitial lung disease (ILD) that may be associated with a progressive fibrosing phenotype. Connective tissue disease (CTD)-ILDs include rheumatoid arthritis-associated ILD, systemic sclerosis-associated ILD, mixed CTD-associated ILD and other autoimmune ILDs. Other ILDs include exposure-related ILDs (asbestosis and silicosis), non-idiopathic pulmonary fibrosis (IPF) idiopathic interstitial pneumonias (desquamative interstitial pneumonia, etc.), and others. g/f PF: genetic and/or familial pulmonary fibrosis; HP: hypersensitivity pneumonitis; iNSIP: idiopathic nonspecific interstitial pneumonia; IPAF: interstitial pneumonia with autoimmune features; uILD: unclassifiable ILD.
FIGURE 2
FIGURE 2
Diagnosis of fibrosing interstitial lung diseases (ILDs) that may present a progressive phenotype. BAL: bronchoalveolar lavage; HRCT: high-resolution computed tomography; MDD: multidisciplinary diagnosis; PF-ILD: progressive-fibrosing ILD; PFT: pulmonary function test. Reproduced from [6].
See this image and copyright information in PMC

Comment on

References

    1. Travis WD, Costabel U, Hansell DM, et al. . An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013; 188: 733–748. - PMC - PubMed
    1. Raghu G, Collard HR, Egan JJ, et al. . An Official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011; 183: 788–824. - PMC - PubMed
    1. Flaherty KR, Brown KK, Wells AU, et al. . Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Respir Res 2017; 4: e000212. - PMC - PubMed
    1. Wells AU, Brown KK, Flaherty KR, et al. . What's in a name? That which we call IPF, by any other name would act the same. Eur Respir J 2018; 51: 1800692. - PubMed
    1. Collins BF, Raghu G. Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis. Eur Respir Rev 2019; 28: 190021. - PMC - PubMed