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J Korean Soc Radiol. 2014 Apr;70(4):255-259. Korean. Case Report. https://doi.org/10.3348/jksr.2014.70.4.255
Park JC , Chae EJ , Song JS .
Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. ejinchae@amc.seoul.kr
Department of Radiology, School of Medicine, Catholic University of Daegu, Daegu, Korea.
Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
Abstract

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described, very rare type of fibrotic interstitial lung disease predominantly involving subpleural areas of both upper lungs. IPPFE has distinctive radiologic and pathologic features: progressive subpleural opacity with fibrotic changes, predominantly in upper lungs, and dense elastic component on histology. We experienced one case of surgically confirmed IPPFE, with progression of radiologic findings on the serial CT examinations. We herein report the characteristic radiologic features of IPPFE with pathologic and clinical manifestations.

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