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KMID : 1141720190070020105
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폐쇄성폐질환
2019 Volume.7 No. 2 p.105 ~ p.110
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Lee Jae-Ha
Song Jin-Woo
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Abstract
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Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia (IIP) characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal fibrosis. After first report in 2004, it was classified as the group of rare IIP in the international consensus classification of IIP in 2013. Although there was no consensus on diagnostic criteria of PPFE, the radiological and histopathological criteria, suggested by Reddy et al. in 2012, had been generally accepted. The etiology is still unclear but often associated with underlying clinical condition including organ transplantation, chemotherapy, autoimmune disease and respiratory infection. Cough and dyspnea are the frequent presenting symptoms and pneumothorax is the most common complication. Low forced vital capacity and total lung capacity (TLC), and increased ratio of residual volume over TLC are characteristic lung function findings. The clinical course of PPFE was reported to be similar to chronic fibrosing interstitial pneumonias of slow progression, but sometimes shows rapid progression. There are increasing evidences for the coexistence of other interstitial lung disease (ILD) in their lower lobe (43∼91.7%) and usual interstitial pneumonia pattern is the most common histopathologic finding. Currently there is no proven pharmacologic treatment and only lung transplantation is considered as definite therapeutic option. Although increased reports and awareness of PPFE, data on clinical characteristics of Korean patients with PPFE is still lacking, and need to be addressed in the future.
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KEYWORD
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Idiopathic interstitial pneumonia, Pleuroparenchymal fibroelastosis, Etiology, Pulmonary function test
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