A Case of Interstitial Lung Disease With Apical Pleural Thickening: Idiopathic Pleuroparenchymal Fibroelastosis

Abstract

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a very rare, recently described condition, characterised by fibrotic thickening of the pleural and subpleural parenchyma, predominantly in the upper lobes.1 Clinical manifestations and lung function tests are similar to those observed in restrictive interstitial pneumonias, and in some of the cases described, there was a history of recurrent infections, such as allergic bronchopulmonary aspergillosis or cystic fibrosis.2,3 Radiological findings include intense pleural thickening associated with signs of fibrosis, particularly in the upper lobes, with loss of volume and structural distortion, as observed in the case presented here.