title: Pleuroparenchymal Fibroelastosis: A New Entity within the Spectrum of Rare Idiopathic Interstitial Pneumonias.
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.
authors.affiliation: Department of Pulmonary Medicine, Hospital Universitari Germans Trias i Pujol, Universitat Autónoma de Barcelona, Badalona, 08916 Catalonia, Spain.
authors.forename: Karina
authors.initials: K
authors.lastname: Portillo
authors.affiliation: Department of Radiology, Hospital Universitari Germans Trias i Pujol, Universitat Autónoma de Barcelona, Badalona, 08916 Catalonia, Spain.
authors.forename: Ignasi
authors.initials: I
authors.lastname: Guasch
authors.affiliation: Department of Pulmonary Medicine, Hospital Universitari Germans Trias i Pujol, Universitat Autónoma de Barcelona, Badalona, 08916 Catalonia, Spain.
authors.forename: Caroline
authors.initials: C
authors.lastname: Becker
authors.affiliation: Department of Pulmonary Medicine, Hospital Universitari Germans Trias i Pujol, Universitat Autónoma de Barcelona, Badalona, 08916 Catalonia, Spain ; CIBER de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Palma de Mallorca, Spain.
authors.forename: Felipe
authors.initials: F
authors.lastname: Andreo
authors.affiliation: Department of Pathology, Hospital Universitari Germans Trias i Pujol, Universitat Autónoma de Barcelona, Badalona, 08916 Catalonia, Spain.
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Fernández-Figueras
authors.affiliation: Department of Pathology, Hospital Clínic, University of Barcelona, Barcelona, 08036 Catalonia, Spain.
authors.forename: José
authors.initials: J
authors.lastname: Ramirez Ruz
authors.affiliation: Department of Thoracic Surgery, Hospital Universitari Germans Trias i Pujol, Universitat Autónoma de Barcelona, Badalona, 08916 Catalonia, Spain.
authors.forename: Carlos
authors.initials: C
authors.lastname: Martinez-Barenys
authors.affiliation: Department of Thoracic Surgery, Hospital Universitari Germans Trias i Pujol, Universitat Autónoma de Barcelona, Badalona, 08916 Catalonia, Spain.
authors.forename: Samuel
authors.initials: S
authors.lastname: García-Reina
authors.affiliation: Department of Thoracic Surgery, Hospital Universitari Germans Trias i Pujol, Universitat Autónoma de Barcelona, Badalona, 08916 Catalonia, Spain.
authors.forename: Pedro
authors.initials: P
authors.lastname: Lopez de Castro
authors.affiliation: Pathology Department, Hospital Universitari Vall d'Hebron, Universitat Autónoma de Barcelona, Barcelona, 08035 Catalonia, Spain.
authors.forename: Irene
authors.initials: I
authors.lastname: Sansano
authors.affiliation: CIBER de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Palma de Mallorca, Spain ; Department of Pulmonary Medicine, Hospital Universitari Vall d'Hebron, Universitat Autónoma de Barcelona, 08035 Catalonia, Spain.
authors.forename: Ana
authors.initials: A
authors.lastname: Villar
authors.affiliation: Department of Pulmonary Medicine, Hospital Universitari Germans Trias i Pujol, Universitat Autónoma de Barcelona, Badalona, 08916 Catalonia, Spain ; CIBER de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Palma de Mallorca, Spain ; Department of Internal Medicine, Universitat Autónoma de Barcelona, Catalonia, Spain.
authors.forename: Juan
authors.initials: J
authors.lastname: Ruiz-Manzano