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Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics

Pleuroparenchymal fibroelastosis (PPFE) is an unusual pulmonary disease with unique clinical, radiological, and pathological characteristics. Designated a rare idiopathic interstitial pneumonia in 2013, its name refers to a combination of fibrosis involving the visceral pleura and fibroelastotic cha...

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Autores principales: Chua, Felix, Desai, Sujal R., Nicholson, Andrew G., Devaraj, Anand, Renzoni, Elisabetta, Rice, Alexandra, Wells, Athol U.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Thoracic Society 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6945468/
https://www.ncbi.nlm.nih.gov/pubmed/31425665
http://dx.doi.org/10.1513/AnnalsATS.201902-181CME
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author Chua, Felix
Desai, Sujal R.
Nicholson, Andrew G.
Devaraj, Anand
Renzoni, Elisabetta
Rice, Alexandra
Wells, Athol U.
author_facet Chua, Felix
Desai, Sujal R.
Nicholson, Andrew G.
Devaraj, Anand
Renzoni, Elisabetta
Rice, Alexandra
Wells, Athol U.
author_sort Chua, Felix
collection PubMed
description Pleuroparenchymal fibroelastosis (PPFE) is an unusual pulmonary disease with unique clinical, radiological, and pathological characteristics. Designated a rare idiopathic interstitial pneumonia in 2013, its name refers to a combination of fibrosis involving the visceral pleura and fibroelastotic changes predominating in the subpleural lung parenchyma. Although a number of disease associations have been described, no single cause of PPFE has been unequivocally identified. A diagnosis of PPFE is most commonly achieved by identifying characteristic abnormalities on computed tomographic scans. The earliest changes are consistently located in the upper lobes close to the lung apices, the same locations where subsequent disease progression is also most conspicuous. When sufficiently severe, the disease leads to progressive volume loss of the upper lobes, which, in combination with decreased body mass, produces platythorax. Once regarded as a slowly progressing entity, it is now acknowledged that some patients with PPFE follow an inexorably progressive course that culminates in irreversible respiratory failure and early death. In the absence of effective medical drug treatment, lung transplant remains the only therapeutic option for this disorder. This review focuses on improving early disease recognition and evaluating its pathophysiological impact and discusses working approaches for its management.
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spelling pubmed-69454682020-01-14 Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics Chua, Felix Desai, Sujal R. Nicholson, Andrew G. Devaraj, Anand Renzoni, Elisabetta Rice, Alexandra Wells, Athol U. Ann Am Thorac Soc Focused Review Pleuroparenchymal fibroelastosis (PPFE) is an unusual pulmonary disease with unique clinical, radiological, and pathological characteristics. Designated a rare idiopathic interstitial pneumonia in 2013, its name refers to a combination of fibrosis involving the visceral pleura and fibroelastotic changes predominating in the subpleural lung parenchyma. Although a number of disease associations have been described, no single cause of PPFE has been unequivocally identified. A diagnosis of PPFE is most commonly achieved by identifying characteristic abnormalities on computed tomographic scans. The earliest changes are consistently located in the upper lobes close to the lung apices, the same locations where subsequent disease progression is also most conspicuous. When sufficiently severe, the disease leads to progressive volume loss of the upper lobes, which, in combination with decreased body mass, produces platythorax. Once regarded as a slowly progressing entity, it is now acknowledged that some patients with PPFE follow an inexorably progressive course that culminates in irreversible respiratory failure and early death. In the absence of effective medical drug treatment, lung transplant remains the only therapeutic option for this disorder. This review focuses on improving early disease recognition and evaluating its pathophysiological impact and discusses working approaches for its management. American Thoracic Society 2019-11 /pmc/articles/PMC6945468/ /pubmed/31425665 http://dx.doi.org/10.1513/AnnalsATS.201902-181CME Text en Copyright © 2019 by the American Thoracic Society http://creativecommons.org/licenses/by-nc-nd/4.0/ This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivatives License 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/). For commercial usage and reprints, please contact Diane Gern (dgern@thoracic.org).
spellingShingle Focused Review
Chua, Felix
Desai, Sujal R.
Nicholson, Andrew G.
Devaraj, Anand
Renzoni, Elisabetta
Rice, Alexandra
Wells, Athol U.
Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics
title Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics
title_full Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics
title_fullStr Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics
title_full_unstemmed Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics
title_short Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics
title_sort pleuroparenchymal fibroelastosis. a review of clinical, radiological, and pathological characteristics
topic Focused Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6945468/
https://www.ncbi.nlm.nih.gov/pubmed/31425665
http://dx.doi.org/10.1513/AnnalsATS.201902-181CME
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