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Fibrosing interstitial lung diseases: knowns and unknowns

Patients with certain types of fibrosing interstitial lung disease (ILD) are at risk of developing a progressive phenotype characterised by self-sustaining fibrosis, decline in lung function, worsening quality of life, and early mortality. It has been proposed that such progressive fibrosing ILDs, w...

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Autores principales: Cottin, Vincent, Wollin, Lutz, Fischer, Aryeh, Quaresma, Manuel, Stowasser, Susanne, Harari, Sergio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: European Respiratory Society 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9489101/
https://www.ncbi.nlm.nih.gov/pubmed/30814139
http://dx.doi.org/10.1183/16000617.0100-2018
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author Cottin, Vincent
Wollin, Lutz
Fischer, Aryeh
Quaresma, Manuel
Stowasser, Susanne
Harari, Sergio
author_facet Cottin, Vincent
Wollin, Lutz
Fischer, Aryeh
Quaresma, Manuel
Stowasser, Susanne
Harari, Sergio
author_sort Cottin, Vincent
collection PubMed
description Patients with certain types of fibrosing interstitial lung disease (ILD) are at risk of developing a progressive phenotype characterised by self-sustaining fibrosis, decline in lung function, worsening quality of life, and early mortality. It has been proposed that such progressive fibrosing ILDs, which show commonalities in clinical behaviour and in the pathogenetic mechanisms that drive progressive fibrosis, may be “lumped” together for the purposes of clinical research and, potentially, for treatment. At present, no drugs are approved for the treatment of ILDs other than nintedanib and pirfenidone for the treatment of idiopathic pulmonary fibrosis. For other progressive fibrosing ILDs, the mainstay of drug therapy is immunosuppression. However, it is postulated that, once the response to lung injury in fibrosing ILDs has reached the stage at which fibrosis has become progressive and self-sustaining, targeted antifibrotic therapy would be required to slow disease progression. Nintedanib, an intracellular inhibitor of tyrosine kinases, has shown antifibrotic, anti-inflammatory and vascular remodelling effects in several non-clinical models of fibrosis, irrespective of the trigger for the injury. Ongoing clinical trials will provide insight into the role of antifibrotic treatment with nintedanib or pirfenidone in the management of fibrosing ILDs with a progressive phenotype.
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spelling pubmed-94891012022-11-14 Fibrosing interstitial lung diseases: knowns and unknowns Cottin, Vincent Wollin, Lutz Fischer, Aryeh Quaresma, Manuel Stowasser, Susanne Harari, Sergio Eur Respir Rev Review Patients with certain types of fibrosing interstitial lung disease (ILD) are at risk of developing a progressive phenotype characterised by self-sustaining fibrosis, decline in lung function, worsening quality of life, and early mortality. It has been proposed that such progressive fibrosing ILDs, which show commonalities in clinical behaviour and in the pathogenetic mechanisms that drive progressive fibrosis, may be “lumped” together for the purposes of clinical research and, potentially, for treatment. At present, no drugs are approved for the treatment of ILDs other than nintedanib and pirfenidone for the treatment of idiopathic pulmonary fibrosis. For other progressive fibrosing ILDs, the mainstay of drug therapy is immunosuppression. However, it is postulated that, once the response to lung injury in fibrosing ILDs has reached the stage at which fibrosis has become progressive and self-sustaining, targeted antifibrotic therapy would be required to slow disease progression. Nintedanib, an intracellular inhibitor of tyrosine kinases, has shown antifibrotic, anti-inflammatory and vascular remodelling effects in several non-clinical models of fibrosis, irrespective of the trigger for the injury. Ongoing clinical trials will provide insight into the role of antifibrotic treatment with nintedanib or pirfenidone in the management of fibrosing ILDs with a progressive phenotype. European Respiratory Society 2019-02-27 /pmc/articles/PMC9489101/ /pubmed/30814139 http://dx.doi.org/10.1183/16000617.0100-2018 Text en Copyright ©ERS 2019. https://creativecommons.org/licenses/by-nc/4.0/ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.
spellingShingle Review
Cottin, Vincent
Wollin, Lutz
Fischer, Aryeh
Quaresma, Manuel
Stowasser, Susanne
Harari, Sergio
Fibrosing interstitial lung diseases: knowns and unknowns
title Fibrosing interstitial lung diseases: knowns and unknowns
title_full Fibrosing interstitial lung diseases: knowns and unknowns
title_fullStr Fibrosing interstitial lung diseases: knowns and unknowns
title_full_unstemmed Fibrosing interstitial lung diseases: knowns and unknowns
title_short Fibrosing interstitial lung diseases: knowns and unknowns
title_sort fibrosing interstitial lung diseases: knowns and unknowns
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9489101/
https://www.ncbi.nlm.nih.gov/pubmed/30814139
http://dx.doi.org/10.1183/16000617.0100-2018
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