Autopsy findings in a case of idiopathic pleuroparenchymal fibroelastosis: A rare entity with review of literature

Abstract

Idiopathic pleuroparenchymal fibroelastosis is a rare idiopathic interstitial pneumonia which mainly affects the upper lobe of lungs resulting in intra alveolar fibrosis, elastosis and visceral pleural fibrosis. For definite diagnosis, radiographic findings as well as histopathology findings are required. The overlap with interstitial lung diseases seen usually, makes it challenging to diagnose. There are no guidelines available for the diagnosis and appropriate management of this disease and only a few cases reported in the literature. We report a case of pleuroparenchymal fibroelastosis in autopsy in a 32-year-old female who allegedly died of heart failure with review of literature.