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Pleuroparenchymal Fibroelastosis : A New Entity within the Spectrum of Rare Idiopathic Interstitial Pneumonias
Portillo, Karina (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Guasch, Ignasi (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Becker, Caroline (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Andreo, Felipe (CIBER de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III)
Fernández-Figueras, Maria Teresa (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Ramirez Ruz, José (Hospital Clínic i Provincial de Barcelona)
Martinez-Barenys, Carlos (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
García-Reina, Samuel (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Lopez de Castro, Pedro (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Sansano, Irene (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Villar, Ana (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Ruiz Manzano, J (Universitat Autònoma de Barcelona. Departament de Medicina)

Date: 2015
Abstract: Pleuroparenchymal fibroelastosis (PPFE) is a rare entity that has been recently included in the official American Thoracic Society/European Respiratory Society (ATS/ERS) statement in 2013 as a group of rare idiopathic interstitial pneumonias (IIPs). PPFE is characterized by pleural and subpleural parenchymal thickening due to elastic fiber proliferation, mainly in the upper lobes. The etiology of the disease is unclear, although some cases have been associated as a complication after bone marrow transplantation, lung transplantation (LT), chemotherapy, and recurrent respiratory infections. The patients usually report progressive dyspnea and dry cough and are predisposed to develop spontaneous or iatrogenic pneumothoraces after surgical lung biopsy (SLB) for its diagnosis. That is why better awareness with the clinical and radiologic features can help optimal management by the multidisciplinary team. Novel invasive techniques such as cryobiopsy may become useful tools in these patients as it could spare SLB. We present the first reported cases in Spain.
Rights: Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan aquestes es distribueixin sota la mateixa llicència que regula l'obra original i es reconegui l'autoria de l'obra original. Creative Commons
Language: Anglès
Document: article ; altres ; publishedVersion
Published in: Case Reports in Pulmonology, Vol. 2015 (august 2015) , ISSN 2090-6854

DOI: 10.1155/2015/810515
PMID: 26380141


9 p, 5.6 MB

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Articles > Research articles
Articles > Published articles

 Record created 2018-01-31, last modified 2021-04-20



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