부산대학교 도서관

Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease, characterized by pleural and subpleural parenchymal fibrosis, predominantly in the upper lobes. Around 60% of patients with PPFE develop spontaneous pneumothorax during the disease course. Previous studies, however, rarely addressed the natural course and duration of pneumothorax in these patients. Here, we reported 2 patients with PPFE who developed spontaneous pneumothorax lasting for more than 6 months. Serial chest images and pulmonary function tests were also provided. It was notable that both patients could still perform the forced expiratory maneuver during spirometry without further lung collapse, probably due to thickening and fibrosis of the visceral pleura and subpleural lung parenchyma. Our experience with these 2 cases suggests that prolonged pneumothorax can develop in patients with PPFE, due to its unique pathophysiology.