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Pleuroparenchymal Fibroelastosis: A New Entity within the Spectrum of Rare Idiopathic Interstitial Pneumonias
Joint Authors
Portillo, Karina
Guasch, Ignasi
Becker, Caroline
Andreo, Felipe
Fernández-Figueras, Maria Teresa
Ramirez Ruz, José
Martinez-Barenys, Carlos
García-Reina, Samuel
Lopez de Castro, Pedro
Sansano, Irene
Villar, Ana
Ruiz-Manzano, Juan
Source
Issue
Vol. 2015, Issue 2015 (31 Dec. 2015), pp.1-9, 9 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2015-08-26
Country of Publication
Egypt
No. of Pages
9
Main Subjects
Abstract EN
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.
American Psychological Association (APA)
Portillo, Karina& Guasch, Ignasi& Becker, Caroline& Andreo, Felipe& Fernández-Figueras, Maria Teresa& Ramirez Ruz, José…[et al.]. 2015. Pleuroparenchymal Fibroelastosis: A New Entity within the Spectrum of Rare Idiopathic Interstitial Pneumonias. Case Reports in Pulmonology،Vol. 2015, no. 2015, pp.1-9.
https://search.emarefa.net/detail/BIM-1059887
Modern Language Association (MLA)
Portillo, Karina…[et al.]. Pleuroparenchymal Fibroelastosis: A New Entity within the Spectrum of Rare Idiopathic Interstitial Pneumonias. Case Reports in Pulmonology No. 2015 (2015), pp.1-9.
https://search.emarefa.net/detail/BIM-1059887
American Medical Association (AMA)
Portillo, Karina& Guasch, Ignasi& Becker, Caroline& Andreo, Felipe& Fernández-Figueras, Maria Teresa& Ramirez Ruz, José…[et al.]. Pleuroparenchymal Fibroelastosis: A New Entity within the Spectrum of Rare Idiopathic Interstitial Pneumonias. Case Reports in Pulmonology. 2015. Vol. 2015, no. 2015, pp.1-9.
https://search.emarefa.net/detail/BIM-1059887
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1059887