Pleuroparenchymal Fibroelastosis: A Case Report

Abstract

Pleuroparenchymal fibroelastosis (PPFE) is a rare, idiopathic interstitial pneumonia. wherein the first symptom might be dyspnea or a dry cough. The condition can also be manifested with chest pain secondary to pneumothorax. While the definitive diagnosis is based on a histological evaluation (which is not often performed), a computed tomography scan shows findings, such as apical fibrosis and pleural thickening of the apical lobes, which help assess the diagnosis. We describe a case of PPFE diagnosed radiologically in a 69-year-old man. This case highlights that PPFE is a pathology that can go unnoticed for a long time, and patients might neglect the revealing symptoms such as coughing.

Keywords: idiopathic interstitial pneumonia; lung biopsy; lung transplantation; pleural thickening; pleuropulmonary elastosis; subpleural fibrosis.

Figure 1. Coronal view of thoracic computed tomography with bone window study (A) and parenchymal window study (B and C) showing apical pleural thickening and subpleural consolidations (yellow arrows), and traction bronchiectasis (red arrow).

Figure 2. A: Axial view thoracic computed tomography showing apical pleural thickening and subpleural consolidations (yellow arrows). B: Axial view thoracic computed tomography showing traction bronchiectasis (red arrow).