Idiopathic Pleuroparenchymal Fibroelastosis Presenting in Recurrent Pneumothorax: A Case Report

• Published in: Tuberculosis and respiratory diseases Vol. 77; no. 4; pp. 184 - 187
• Main Authors: Noh, Hyun Jin, Seo, Yun, Huo, Sol Mi, Kim, Tae Jung, Kim, Hyo Lim, Song, Jeong Sup
• Format: Journal Article
• Language: English
• Published: Korea (South) The Korean Academy of Tuberculosis and Respiratory Diseases 01.10.2014; 대한결핵및호흡기학회
• Subjects: Case Report; 내과학; Pneumothorax; Idiopathic Interstitial Pneumonias; Fibrosis
• ISSN: 1738-3536; 2005-6184
• DOI: 10.4046/trd.2014.77.4.184

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.