Pleuroparenchymal Fibroelastosis: A Rare Interstitial Lung Disease

Abstract

Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease (ILD) characterized by upper-lobe predominant fibrosis involving of the pleural and subpleural lung parenchyma. It is a relatively newly recognized entity within the spectrum of ILDs. Diagnosing PPFE can be challenging due to its rarity and overlapping clinical features with other ILDs. We report the case of two patients, siblings, of first degree consanguineous parents, 37 and 32 years old, suffering from worsening dyspnea, dry cough and weight loss, the high resolution chest computer tomography objectified a platythorax, a bilateral pleural cap thickening, diffuse sub pleural reticulations with bilateral apical traction bronchiectasis, associated with septal and non-septal lines, the main scannographic lesions spared the lower lobes. After eliminating differential diagnoses, the diagnosis of PPFE in a familial form was made based on the radiological criteria proposed by Reddy and al and on according to the 2013 ATS/ERS consensus. We underline the importance of reporting these 2 cases to learn more about the disease, a rare chronic fibrosing interstitial pneumonia, and its serious prognosis in the absence of therapies.