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Interstitial pneumonia with autoimmune features: challenges and controversies

The presence of clinical, serological and/or radiological features suggestive, but not confirmatory, of a defined connective tissue disease in patients with interstitial lung disease is a relatively frequent occurrence. In 2015, the European Respiratory Society and the American Thoracic Society prop...

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Autores principales: Mackintosh, John A., Wells, Athol U., Cottin, Vincent, Nicholson, Andrew G., Renzoni, Elisabetta A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: European Respiratory Society 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9488577/
https://www.ncbi.nlm.nih.gov/pubmed/34937706
http://dx.doi.org/10.1183/16000617.0177-2021
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author Mackintosh, John A.
Wells, Athol U.
Cottin, Vincent
Nicholson, Andrew G.
Renzoni, Elisabetta A.
author_facet Mackintosh, John A.
Wells, Athol U.
Cottin, Vincent
Nicholson, Andrew G.
Renzoni, Elisabetta A.
author_sort Mackintosh, John A.
collection PubMed
description The presence of clinical, serological and/or radiological features suggestive, but not confirmatory, of a defined connective tissue disease in patients with interstitial lung disease is a relatively frequent occurrence. In 2015, the European Respiratory Society and the American Thoracic Society proposed classification criteria for the interstitial pneumonia with autoimmune features (IPAF) research entity to capture such patients in a standardised manner, with the intention of nurturing clinical research. This initiative resulted in the publication of several series of IPAF patients, with significant variation between cohorts in clinical characteristics, outcome and the application of IPAF criteria in patient selection. From this increasing body of published work, it has become apparent that revision of IPAF criteria is now required in order to justify the eventual designation of IPAF as a standalone diagnostic term, as opposed to a provisional entity put forward as a basis for clinical research. This review covers the current state of IPAF, conclusions that can and cannot be drawn from the IPAF evidence base, and ongoing uncertainties that require further expert group consideration.
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spelling pubmed-94885772022-11-14 Interstitial pneumonia with autoimmune features: challenges and controversies Mackintosh, John A. Wells, Athol U. Cottin, Vincent Nicholson, Andrew G. Renzoni, Elisabetta A. Eur Respir Rev Reviews The presence of clinical, serological and/or radiological features suggestive, but not confirmatory, of a defined connective tissue disease in patients with interstitial lung disease is a relatively frequent occurrence. In 2015, the European Respiratory Society and the American Thoracic Society proposed classification criteria for the interstitial pneumonia with autoimmune features (IPAF) research entity to capture such patients in a standardised manner, with the intention of nurturing clinical research. This initiative resulted in the publication of several series of IPAF patients, with significant variation between cohorts in clinical characteristics, outcome and the application of IPAF criteria in patient selection. From this increasing body of published work, it has become apparent that revision of IPAF criteria is now required in order to justify the eventual designation of IPAF as a standalone diagnostic term, as opposed to a provisional entity put forward as a basis for clinical research. This review covers the current state of IPAF, conclusions that can and cannot be drawn from the IPAF evidence base, and ongoing uncertainties that require further expert group consideration. European Respiratory Society 2021-12-22 /pmc/articles/PMC9488577/ /pubmed/34937706 http://dx.doi.org/10.1183/16000617.0177-2021 Text en Copyright ©The authors 2021 https://creativecommons.org/licenses/by-nc/4.0/This version is distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0. For commercial reproduction rights and permissions contact permissions@ersnet.org (mailto:permissions@ersnet.org)
spellingShingle Reviews
Mackintosh, John A.
Wells, Athol U.
Cottin, Vincent
Nicholson, Andrew G.
Renzoni, Elisabetta A.
Interstitial pneumonia with autoimmune features: challenges and controversies
title Interstitial pneumonia with autoimmune features: challenges and controversies
title_full Interstitial pneumonia with autoimmune features: challenges and controversies
title_fullStr Interstitial pneumonia with autoimmune features: challenges and controversies
title_full_unstemmed Interstitial pneumonia with autoimmune features: challenges and controversies
title_short Interstitial pneumonia with autoimmune features: challenges and controversies
title_sort interstitial pneumonia with autoimmune features: challenges and controversies
topic Reviews
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9488577/
https://www.ncbi.nlm.nih.gov/pubmed/34937706
http://dx.doi.org/10.1183/16000617.0177-2021
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