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Pleuroparenchymal fibroelastosis in heterogeneous clinical conditions: Clinicopathologic analysis of 7 cases

Authors
 Yoon Jin Cha  ;  Joungho Han  ;  Man Pyo Chung  ;  Tae Jung Kim  ;  Sumin Shin 
Citation
 Clinical Respiratory Journal, Vol.12(4) : 1495-1502, 2018 
Journal Title
 Clinical Respiratory Journal 
ISSN
 1752-6981 
Issue Date
2018
Keywords
fibroelastosis ; interstitial lung disease ; pathology ; pleural fibrosis
Abstract
BACKGROUND AND AIMS: Pleuroparenchymal fibroelastosis (PPFE) is classified as a rare idiopathic interstitial pneumonia, and its clinicopathologic features remain unclear. We sought to investigate the clinical, radiologic, and pathologic features of patients with histologically proven PPFE. METHODS: Seven patients who had histologic features of PPFE that were definitive or consistent with PPFE were included. Clinical data and radiologic findings of chest computed tomography were obtained. Histologic evaluation was performed by 2 pathologists. RESULTS: Four patients had elevated serum autoantibodies, 3 had a history of organ transplantation, and 1 had acute Aspergillus infection. All patients had various degrees of restrictive patterns on pulmonary function tests. Histologic examination revealed a variable extent of PPFE. Fibroblastic foci, patchy lymphocytic infiltration, and pneumothorax were observed, but these findings were nonspecific. Radiologically, PPFE lesions were distributed diffusely, rather than being limited to the upper lungs. CONCLUSIONS: PPFE seems to be a histologic finding that may partly overlap with other chronic lung diseases. There can be various underlying clinical states.
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/162432
Full Text
https://onlinelibrary.wiley.com/doi/abs/10.1111/crj.12696
DOI
10.1111/crj.12696
Appears in Collections:
1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실)
Yonsei Authors
차윤진(Cha, Yoon Jin) ORCID logo https://orcid.org/0000-0002-5967-4064
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