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High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: Features and differential diagnosis

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described clinical-pathologic entity characterized by pleural and subpleural parenchymal fibrosis, mainly in the upper lobes. As this disease is extremely rare (only 7 cases have been described in the literature to date) poorly define...

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Autores principales: Piciucchi, Sara, Tomassetti, Sara, Casoni, Gianluca, Sverzellati, Nicola, Carloni, Angelo, Dubini, Alessandra, Gavelli, Giampaolo, Cavazza, Alberto, Chilosi, Marco, Poletti, Venerino
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3197499/
https://www.ncbi.nlm.nih.gov/pubmed/21861891
http://dx.doi.org/10.1186/1465-9921-12-111
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author Piciucchi, Sara
Tomassetti, Sara
Casoni, Gianluca
Sverzellati, Nicola
Carloni, Angelo
Dubini, Alessandra
Gavelli, Giampaolo
Cavazza, Alberto
Chilosi, Marco
Poletti, Venerino
author_facet Piciucchi, Sara
Tomassetti, Sara
Casoni, Gianluca
Sverzellati, Nicola
Carloni, Angelo
Dubini, Alessandra
Gavelli, Giampaolo
Cavazza, Alberto
Chilosi, Marco
Poletti, Venerino
author_sort Piciucchi, Sara
collection PubMed
description Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described clinical-pathologic entity characterized by pleural and subpleural parenchymal fibrosis, mainly in the upper lobes. As this disease is extremely rare (only 7 cases have been described in the literature to date) poorly defined cases of IPPFE can go unrecognized. The clinical course of disease is progressive and prognosis is poor, with no therapeutic options other than lung transplantation currently available, yet. The aim of this report is to describe two further cases of this rare disease, reviewing CT, clinical and histological features.
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spelling pubmed-31974992011-10-21 High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: Features and differential diagnosis Piciucchi, Sara Tomassetti, Sara Casoni, Gianluca Sverzellati, Nicola Carloni, Angelo Dubini, Alessandra Gavelli, Giampaolo Cavazza, Alberto Chilosi, Marco Poletti, Venerino Respir Res Letter to the Editor Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described clinical-pathologic entity characterized by pleural and subpleural parenchymal fibrosis, mainly in the upper lobes. As this disease is extremely rare (only 7 cases have been described in the literature to date) poorly defined cases of IPPFE can go unrecognized. The clinical course of disease is progressive and prognosis is poor, with no therapeutic options other than lung transplantation currently available, yet. The aim of this report is to describe two further cases of this rare disease, reviewing CT, clinical and histological features. BioMed Central 2011 2011-08-23 /pmc/articles/PMC3197499/ /pubmed/21861891 http://dx.doi.org/10.1186/1465-9921-12-111 Text en Copyright ©2011 S et al.; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Letter to the Editor
Piciucchi, Sara
Tomassetti, Sara
Casoni, Gianluca
Sverzellati, Nicola
Carloni, Angelo
Dubini, Alessandra
Gavelli, Giampaolo
Cavazza, Alberto
Chilosi, Marco
Poletti, Venerino
High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: Features and differential diagnosis
title High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: Features and differential diagnosis
title_full High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: Features and differential diagnosis
title_fullStr High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: Features and differential diagnosis
title_full_unstemmed High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: Features and differential diagnosis
title_short High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: Features and differential diagnosis
title_sort high resolution ct and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis
topic Letter to the Editor
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3197499/
https://www.ncbi.nlm.nih.gov/pubmed/21861891
http://dx.doi.org/10.1186/1465-9921-12-111
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