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Idiopathic pulmonary fibrosis: Current and future treatment

OBJECTIVES: Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic lung disease characterized by dry cough, fatigue, and progressive exertional dyspnea. Lung parenchyma and architecture is destroyed, compliance is lost, and gas exchange is compromised in this debilitating condition that leads ine...

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Autores principales: Glass, Daniel S., Grossfeld, David, Renna, Heather A., Agarwala, Priya, Spiegler, Peter, DeLeon, Joshua, Reiss, Allison B.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9060042/
https://www.ncbi.nlm.nih.gov/pubmed/35001525
http://dx.doi.org/10.1111/crj.13466
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author Glass, Daniel S.
Grossfeld, David
Renna, Heather A.
Agarwala, Priya
Spiegler, Peter
DeLeon, Joshua
Reiss, Allison B.
author_facet Glass, Daniel S.
Grossfeld, David
Renna, Heather A.
Agarwala, Priya
Spiegler, Peter
DeLeon, Joshua
Reiss, Allison B.
author_sort Glass, Daniel S.
collection PubMed
description OBJECTIVES: Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic lung disease characterized by dry cough, fatigue, and progressive exertional dyspnea. Lung parenchyma and architecture is destroyed, compliance is lost, and gas exchange is compromised in this debilitating condition that leads inexorably to respiratory failure and death within 3–5 years of diagnosis. This review discusses treatment approaches to IPF in current use and those that appear promising for future development. DATA SOURCE: The data were obtained from the Randomized Controlled Trials and scientific studies published in English literature. We used search terms related to IPF, antifibrotic treatment, lung transplant, and management. RESULTS: Etiopathogenesis of IPF is not fully understood, and treatment options are limited. Pathological features of IPF include extracellular matrix remodeling, fibroblast activation and proliferation, immune dysregulation, cell senescence, and presence of aberrant basaloid cells. The mainstay therapies are the oral antifibrotic drugs pirfenidone and nintedanib, which can improve quality of life, attenuate symptoms, and slow disease progression. Unilateral or bilateral lung transplantation is the only treatment for IPF shown to increase life expectancy. CONCLUSION: Clearly, there is an unmet need for accelerated research into IPF mechanisms so that progress can be made in therapeutics toward the goals of increasing life expectancy, alleviating symptoms, and improving well‐being.
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spelling pubmed-90600422022-07-12 Idiopathic pulmonary fibrosis: Current and future treatment Glass, Daniel S. Grossfeld, David Renna, Heather A. Agarwala, Priya Spiegler, Peter DeLeon, Joshua Reiss, Allison B. Clin Respir J Review Articles OBJECTIVES: Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic lung disease characterized by dry cough, fatigue, and progressive exertional dyspnea. Lung parenchyma and architecture is destroyed, compliance is lost, and gas exchange is compromised in this debilitating condition that leads inexorably to respiratory failure and death within 3–5 years of diagnosis. This review discusses treatment approaches to IPF in current use and those that appear promising for future development. DATA SOURCE: The data were obtained from the Randomized Controlled Trials and scientific studies published in English literature. We used search terms related to IPF, antifibrotic treatment, lung transplant, and management. RESULTS: Etiopathogenesis of IPF is not fully understood, and treatment options are limited. Pathological features of IPF include extracellular matrix remodeling, fibroblast activation and proliferation, immune dysregulation, cell senescence, and presence of aberrant basaloid cells. The mainstay therapies are the oral antifibrotic drugs pirfenidone and nintedanib, which can improve quality of life, attenuate symptoms, and slow disease progression. Unilateral or bilateral lung transplantation is the only treatment for IPF shown to increase life expectancy. CONCLUSION: Clearly, there is an unmet need for accelerated research into IPF mechanisms so that progress can be made in therapeutics toward the goals of increasing life expectancy, alleviating symptoms, and improving well‐being. John Wiley and Sons Inc. 2022-01-10 /pmc/articles/PMC9060042/ /pubmed/35001525 http://dx.doi.org/10.1111/crj.13466 Text en © 2022 The Authors. The Clinical Respiratory Journal published by John Wiley & Sons Ltd. https://creativecommons.org/licenses/by/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review Articles
Glass, Daniel S.
Grossfeld, David
Renna, Heather A.
Agarwala, Priya
Spiegler, Peter
DeLeon, Joshua
Reiss, Allison B.
Idiopathic pulmonary fibrosis: Current and future treatment
title Idiopathic pulmonary fibrosis: Current and future treatment
title_full Idiopathic pulmonary fibrosis: Current and future treatment
title_fullStr Idiopathic pulmonary fibrosis: Current and future treatment
title_full_unstemmed Idiopathic pulmonary fibrosis: Current and future treatment
title_short Idiopathic pulmonary fibrosis: Current and future treatment
title_sort idiopathic pulmonary fibrosis: current and future treatment
topic Review Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9060042/
https://www.ncbi.nlm.nih.gov/pubmed/35001525
http://dx.doi.org/10.1111/crj.13466
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