An adolescent with idiopathic pleuroparenchymal fibroelastosis. Case report


Atag E., Ikizoglu N. B. , Gökdemir Y. , Erdem Eralp E. , Kıyan G. , Yilmazbayhan D., ...More

ARCHIVOS ARGENTINOS DE PEDIATRIA, vol.116, no.1, 2018 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 116 Issue: 1
  • Publication Date: 2018
  • Doi Number: 10.5546/aap.2018.eng.e147
  • Title of Journal : ARCHIVOS ARGENTINOS DE PEDIATRIA

Abstract

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disorder recently included in rare idiopathic interstitial pneumonias according to the updated American Thoracic Society/European Respiratory Society classification. IPPFE is characterized by pleural and subpleural parenchymal fibrosis causing volume loss predominantly in the upper lung lobes. Age of onset is variable, IPPFE mainly occurs in third and fourth decades.