Idiopathic Pleuroparenchymal Fibroelastosis as a Rare Entity within Idiopathic Interstitial Pneumonias

Pleuroparenchymal fibroelastosis (PPFE) is an upper lobe predominant progressive fibrosis with specific pathologic findings including visceral pleural thickening, subpleural elastosis, and intraalveolar collagenous fibrosis. 

PPFE is now recognized by the American Thoracic Society (ATS)/European Respiratory Society (ERS) in their most recent statement on idiopathic interstitial pneumonia in the category of rare idiopathic interstitial pneumonias. Many of the PPFE cases are associated with underlying conditions but the majority is idiopathic. We aimed to present the clinical, radiological and histopathological features of PPFE in a case diagnosed as IPPFE. Read more>>>>>>>>>>>>