Idiopathic pleuroparenchymal fibroelastosis: A rare but increasingly recognized entity
M.T.. Redondoa,, , N.. Meloa, P.C.. Motaa,b, J.M.. Jesusc, C.S.. Mourab,d, S.. Guimarãesd, A.. Moraisa,b
a Department of Pneumology, Centro Hospitalar de São João, Portugal
b Faculty of Medicine of University of Porto, Portugal
c Department of Radiology, Centro Hospitalar de São João, Portugal
d Department of Pathology, Centro Hospitalar de São João, Portugal

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described rare entity, characterized by pleural and subpleural parenchymal fibrosis and elastosis mainly in the upper lobes. The etiology and pathophysiology are unknown. The prognosis is poor, with no effective therapies other than lung transplantation. IPPFE should be properly identified so that it can be approached correctly. This report describes two clinical cases with clinical imaging and histological features compatible with IPPFE.

Interstitial lung disease, Pleuroparenchymal fibrosis, Fibroelastosis


  • Impact Factor: 1.560(2016)
  • 5-years Impact Factor: 1,100
  • SCImago Journal Rank (SJR):0,29
  • Source Normalized Impact per Paper (SNIP):0,685