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A contemporary practical approach to the multidisciplinary management of unclassifiable interstitial lung disease

Fibrotic interstitial lung diseases (ILDs) frequently have nonspecific and overlapping clinical and radiological features, resulting in ∼10–20% of patients with ILD lacking a clear diagnosis and thus being labelled with unclassifiable ILD. The objective of this review is to describe how patients wit...

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Detalles Bibliográficos
Autores principales: Ryerson, Christopher J., Corte, Tamera J., Myers, Jeffrey L., Walsh, Simon L.F., Guler, Sabina A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: European Respiratory Society 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8674517/
https://www.ncbi.nlm.nih.gov/pubmed/34140296
http://dx.doi.org/10.1183/13993003.00276-2021
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author Ryerson, Christopher J.
Corte, Tamera J.
Myers, Jeffrey L.
Walsh, Simon L.F.
Guler, Sabina A.
author_facet Ryerson, Christopher J.
Corte, Tamera J.
Myers, Jeffrey L.
Walsh, Simon L.F.
Guler, Sabina A.
author_sort Ryerson, Christopher J.
collection PubMed
description Fibrotic interstitial lung diseases (ILDs) frequently have nonspecific and overlapping clinical and radiological features, resulting in ∼10–20% of patients with ILD lacking a clear diagnosis and thus being labelled with unclassifiable ILD. The objective of this review is to describe how patients with unclassifiable ILD should be evaluated, and what impact specific clinical, radiological and histopathological features may have on management decisions, focusing on patients with a predominantly fibrotic phenotype. We highlight recent data that have suggested an increasing role for antifibrotic medications in a variety of fibrotic ILDs, but justify the ongoing importance of making an accurate ILD diagnosis given the benefit of immunomodulatory therapies in many patient populations. We provide a practical approach to support management decisions that can be used by clinicians and tested by clinical researchers, and further identify the need for additional research to support a rational and standardised approach to the management of patients with unclassifiable ILD.
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spelling pubmed-86745172021-12-17 A contemporary practical approach to the multidisciplinary management of unclassifiable interstitial lung disease Ryerson, Christopher J. Corte, Tamera J. Myers, Jeffrey L. Walsh, Simon L.F. Guler, Sabina A. Eur Respir J Reviews Fibrotic interstitial lung diseases (ILDs) frequently have nonspecific and overlapping clinical and radiological features, resulting in ∼10–20% of patients with ILD lacking a clear diagnosis and thus being labelled with unclassifiable ILD. The objective of this review is to describe how patients with unclassifiable ILD should be evaluated, and what impact specific clinical, radiological and histopathological features may have on management decisions, focusing on patients with a predominantly fibrotic phenotype. We highlight recent data that have suggested an increasing role for antifibrotic medications in a variety of fibrotic ILDs, but justify the ongoing importance of making an accurate ILD diagnosis given the benefit of immunomodulatory therapies in many patient populations. We provide a practical approach to support management decisions that can be used by clinicians and tested by clinical researchers, and further identify the need for additional research to support a rational and standardised approach to the management of patients with unclassifiable ILD. European Respiratory Society 2021-12-16 /pmc/articles/PMC8674517/ /pubmed/34140296 http://dx.doi.org/10.1183/13993003.00276-2021 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
Ryerson, Christopher J.
Corte, Tamera J.
Myers, Jeffrey L.
Walsh, Simon L.F.
Guler, Sabina A.
A contemporary practical approach to the multidisciplinary management of unclassifiable interstitial lung disease
title A contemporary practical approach to the multidisciplinary management of unclassifiable interstitial lung disease
title_full A contemporary practical approach to the multidisciplinary management of unclassifiable interstitial lung disease
title_fullStr A contemporary practical approach to the multidisciplinary management of unclassifiable interstitial lung disease
title_full_unstemmed A contemporary practical approach to the multidisciplinary management of unclassifiable interstitial lung disease
title_short A contemporary practical approach to the multidisciplinary management of unclassifiable interstitial lung disease
title_sort contemporary practical approach to the multidisciplinary management of unclassifiable interstitial lung disease
topic Reviews
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8674517/
https://www.ncbi.nlm.nih.gov/pubmed/34140296
http://dx.doi.org/10.1183/13993003.00276-2021
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