title: Idiopathic pleuroparenchymal fibroelastosis presenting in recurrent pneumothorax: a case report.
abstract: Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare, recently classified entity that consists of pleural and subjacent parenchymal fibrosis predominantly in the upper lungs. In an official American Thoracic Society/European Respiratory Society statement in 2013, this disease is introduced as a group of rare idiopathic interstitial pneumonias. We describe a case of a 76-year-old woman with cough and recurrent pneumothorax. She was admitted to our hospital with severe cough at first. High resolution computed tomography (HRCT) disclosed multifocal subpleural consolidations with reticular opacities in both lungs, primarily in the upper lobes, suggesting interstitial pneumonia. Rheumatoid lung was diagnosed initially through an elevated rheumatoid factor, HRCT and surgical biopsy at the right lower lobe. However, one month later, pneumothorax recurred. Surgical biopsy was performed at the right upper lobe at this time. The specimens revealed typical subpleural fibroelastosis. We report this as a first case of idiopathic PPFE in Korea after reviewing the symptoms, imaging and pathologic findings.
authors.affiliation: Department of Internal Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea.
authors.forename: Hyun Jin
authors.initials: HJ
authors.lastname: Noh
authors.affiliation: Department of Internal Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea.
authors.forename: Yun
authors.initials: Y
authors.lastname: Seo
authors.affiliation: Department of Internal Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea.
authors.forename: Sol Mi
authors.initials: SM
authors.lastname: Huo
authors.affiliation: Department of Pathology, The Catholic University of Korea College of Medicine, Seoul, Korea.
authors.forename: Tae Jung
authors.initials: TJ
authors.lastname: Kim
authors.affiliation: Department of Radiology, The Catholic University of Korea College of Medicine, Seoul, Korea.
authors.forename: Hyo Lim
authors.initials: HL
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea.
authors.forename: Jeong Sup
authors.initials: JS
authors.lastname: Song
keywords: Fibrosis; Idiopathic Interstitial Pneumonias; Pneumothorax