title: Idiopathic pleuroparenchymal fibroelastosis, a new idiopathic interstitial pneumonia: A case report.
abstract: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease, idiopathic in most of the cases described in the literature. We report the case of a 55-year-old patient, non-smoker with tuberculosis treated in his youth, starting with progressive dyspnoea and cough, with radiographic abnormalities consisting of pleural thickening, bronchiectasis and structural distortion predominantly in the upper lobes. Due to functional impairment and persistent radiographic abnormalities, biopsy by video-assisted thoracoscopic surgical was decided. The presence of striking elastosis, absence of interstitial damage and abrupt boundary of the fibrous lesion with healthy lung allowed the diagnosis of IPPFE. Currently, the patient has no specific treatment and is in follow-up in the Transplant Unit.
authors.affiliation: Department of Respiratory Medicine. La Serranía Hospital, Ronda (Málaga), Spain enriquesoto40@hotmail.com.
authors.forename: Enrique Javier Soto
authors.initials: EJ
authors.lastname: Hurtado
authors.affiliation: Department of Internal Medicine. La Serranía Hospital, Ronda (Málaga), Spain.
authors.forename: Maria Luisa Amaya
authors.initials: ML
authors.lastname: González
authors.affiliation: Department of Pathology. Carlos Haya Hospital. Málaga, Spain.
authors.forename: Maria Del Mar Elena
authors.initials: Mdel M
authors.lastname: Soto
authors.affiliation: Department of Respiratory Medicine. La Serranía Hospital, Ronda (Málaga), Spain.
authors.forename: Francisco Jose Cabello
authors.initials: FJ
authors.lastname: Rueda
authors.affiliation: Department of Radiology. La Serranía Hospital, Ronda (Málaga), Spain.
authors.forename: Francisco Javier Pérez
authors.initials: FJ
authors.lastname: Nadal
authors.affiliation: Department of Internal Medicine. La Serranía Hospital, Ronda (Málaga), Spain.
authors.forename: Alberto Ruíz
authors.initials: AR
authors.lastname: Cantero
mesh_terms: D001706:Biopsy; D003937:Diagnosis, Differential; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D054988:Idiopathic Interstitial Pneumonias / Q000175:diagnosis*; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis* / Q000503:physiopathology; D008168:Lung / Q000000981:diagnostic imaging* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed; D014797:Vital Capacity / Q000502:physiology
keywords: Idiopathic pleuroparenchymal fibroelastosis; idiopathic interstitial pneumonia; pleural fibrosis; pleuroparenchymal fibroelastosis; pulmonary fibrosis
references.citation: Eur Respir J. 2014 Aug;44(2):289-96
references.pmid: 25082907
references.citation: BMC Pulm Med. 2012 Dec 05;12 :72
references.pmid: 23216996
references.citation: Mod Pathol. 2008 Jun;21(6):784-7
references.pmid: 18408658
references.citation: Lung. 2014 Oct;192(5):711-7
references.pmid: 24880792
references.citation: Rev Port Pneumol (2006). 2015 Jan-Feb;21(1):41-4
references.pmid: 25854135
references.citation: Ann Thorac Surg. 2014 Nov;98(5):e115-7
references.pmid: 25441830
references.citation: Mayo Clin Proc. 2003 May;78(5):630-4
references.pmid: 12744552
references.citation: Curr Respir Med Rev. 2013 Aug;9(4):238-247
references.pmid: 24634642
references.citation: J Bronchology Interv Pulmonol. 2011 Jan;18(1):65-8
references.pmid: 23169023
references.citation: Eur Respir J. 1999 Oct;14(4):971-3
references.pmid: 10573252
references.citation: Eur Respir J. 2013 Jan;41(1):243-5
references.pmid: 23277524
references.citation: Respir Med Case Rep. 2014 Mar 14;12:7-9
references.pmid: 26029526
references.citation: Am J Respir Crit Care Med. 2013 Sep 15;188(6):733-48
references.pmid: 24032382
references.citation: Respir Res. 2011 Aug 23;12 :111
references.pmid: 21861891
references.citation: Eur Respir J. 2014 Aug;44(2):523-7
references.pmid: 24791830
references.citation: Chest. 2004 Dec;126(6):2007-13
references.pmid: 15596706
references.citation: BMC Pulm Med. 2014 May 28;14 :91
references.pmid: 24886550