Idiopathic pleuroparenchymatous fibroelastosis: A case report and brief review of the literature

We describe a patient with idiopathic pleuroparenchymal fibroelastosis (IPPFE). This rare clinicopathological syndrome is characterized by typical apical alterations op chest imaging, such as pleural thickening and subpleural fibrosis. Thickened visceral pleura and subpleural fibrosis consisting of...

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Autores principales: Cuppens, Kristof, Verbeken, Eric, Coolen, Johan, Verschakelen, Johny, Wuyts, Wim
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2014
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4061435/
https://www.ncbi.nlm.nih.gov/pubmed/26029526
http://dx.doi.org/10.1016/j.rmcr.2013.12.005
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author Cuppens, Kristof
Verbeken, Eric
Coolen, Johan
Verschakelen, Johny
Wuyts, Wim
author_facet Cuppens, Kristof
Verbeken, Eric
Coolen, Johan
Verschakelen, Johny
Wuyts, Wim
author_sort Cuppens, Kristof
collection PubMed
description We describe a patient with idiopathic pleuroparenchymal fibroelastosis (IPPFE). This rare clinicopathological syndrome is characterized by typical apical alterations op chest imaging, such as pleural thickening and subpleural fibrosis. Thickened visceral pleura and subpleural fibrosis consisting of dense collagen and elastin, are the main histopathological features. Etiology is unknown but a link between recurrent infections (in particular aspergillosis) and autoimmune diseases is suspected. At this time there is no standardized treatment regimen and the prognosis is variable.
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spelling pubmed-40614352014-10-15 Idiopathic pleuroparenchymatous fibroelastosis: A case report and brief review of the literature Cuppens, Kristof Verbeken, Eric Coolen, Johan Verschakelen, Johny Wuyts, Wim Respir Med Case Rep Case Report We describe a patient with idiopathic pleuroparenchymal fibroelastosis (IPPFE). This rare clinicopathological syndrome is characterized by typical apical alterations op chest imaging, such as pleural thickening and subpleural fibrosis. Thickened visceral pleura and subpleural fibrosis consisting of dense collagen and elastin, are the main histopathological features. Etiology is unknown but a link between recurrent infections (in particular aspergillosis) and autoimmune diseases is suspected. At this time there is no standardized treatment regimen and the prognosis is variable. Elsevier 2014-03-14 /pmc/articles/PMC4061435/ /pubmed/26029526 http://dx.doi.org/10.1016/j.rmcr.2013.12.005 Text en © 2014 The Authors http://creativecommons.org/licenses/by-nc-nd/3.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/3.0/).
spellingShingle Case Report
Cuppens, Kristof
Verbeken, Eric
Coolen, Johan
Verschakelen, Johny
Wuyts, Wim
Idiopathic pleuroparenchymatous fibroelastosis: A case report and brief review of the literature
title Idiopathic pleuroparenchymatous fibroelastosis: A case report and brief review of the literature
title_full Idiopathic pleuroparenchymatous fibroelastosis: A case report and brief review of the literature
title_fullStr Idiopathic pleuroparenchymatous fibroelastosis: A case report and brief review of the literature
title_full_unstemmed Idiopathic pleuroparenchymatous fibroelastosis: A case report and brief review of the literature
title_short Idiopathic pleuroparenchymatous fibroelastosis: A case report and brief review of the literature
title_sort idiopathic pleuroparenchymatous fibroelastosis: a case report and brief review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4061435/
https://www.ncbi.nlm.nih.gov/pubmed/26029526
http://dx.doi.org/10.1016/j.rmcr.2013.12.005
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