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Clinical Features and Prognosis of Pleuroparenchymal Fibroelastosis in Korean
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A1622 - Clinical Features and Prognosis of Pleuroparenchymal Fibroelastosis in Korean
Author Block: J. Lee1, J. Song2, D. Kim3; 1Haeundae Paik Hospital, Inje University College of Medicine, Busan, Korea, Republic of, 2Asan Medical Center, Seoul, Korea, Republic of, 3Asan medical center, Seoul, Korea, Republic of.
Background: Pleuroparenchymal fibroelastosis (PPFE) is one of rare idiopathic interstitial pneumonias (IIP); however, clinical features and prognosis of Korean patients are not well defined.
Methods: A total of 26 patients, who were confirmed by lung biopsy (surgical lung biopsy: 15, transbronchial lung biopsy: 11) from 2010 to 2017 at Asan Medical Center, Seoul, Korea, were included and clinical data were retrospectively analyzed.
Results: Median follow-up period was 13.5 months. Mean age was 62.5±9.9 years old, 61.5% were men and 55% were smokers. Cough (69.2%) and dyspnea (80.8%) were the most frequent presenting symptoms. About half of the subjects had a history of pulmonary infection (42.3%), chemotherapy (11.5%) and organ transplantation (7.7%). Most patients (73.1%) showed restrictive pattern on lung function test (forced vital caapcity: 61.8±19.5% predicted, diffusing capacity of the lung for carbon monoxide: 55.3±18.4% predicted) and neutrophilia (14.5±17.7%) on bronchoalveolar larvage. In 65.4% of subjects, other interstitial lung disease (ILD) was also found in their lower lobes on chest computed tomography (CT) scan images, and in patients whose lower lobe was biopsied (n=15), usual interstitial pneumonia (UIP) pattern was the most common (40%) and fibroelastosis was found in 33.3%. Pneumothorax was developed in 26.9%. Eighteen (69.2%) patients were treated with steroid±immunosuppresants, but 15.4% died during follow-up (1-year survival rate: 89.2%, 2-year: 82.8%), mostly due to respiratory infection (75%). Subjects with lower-lobe involvement on chest CT scan images, showed older age (65.8±20.7 vs 56.3±18.9; p=0.066), lower residual volume (62.9±19.2 vs. 82.7±12.1; p=0.025) and shorter distance during six minute walk test (411.7±94.0 vs. 513.8±95.4; p=0.009), compared to those without; however, survival was similar (median survival period: seventeen months vs. twenty months; p=0.978 ).
Conclusions: About half of Korean PPFE patients had a relevant exposure history and two thirds of them had different ILD in their lower lobes, UIP pattern being the most common. Pneumothorax was common and survival was similar to other IIPs.
Key word : Pleuroparenchymal fibroelastosis, clinical features, prognosis
Table 1. Comparison of baseline characteristics between PPFE subjects with and without lower-lobe involvement.
Data are presented as No. (%) unless indicated otherwise
a BMI, body mass index; b FVC, forced vital capacity; FEV1, forced expiratory volume 1 second; DLco, diffusing capacity of the lungs for carbon monoxide; TLC, total lung capacity; RV, residual volume
c RVSP, right ventricular systolic pressure; d BAL, bronchoalveolar lavage
Author Block: J. Lee1, J. Song2, D. Kim3; 1Haeundae Paik Hospital, Inje University College of Medicine, Busan, Korea, Republic of, 2Asan Medical Center, Seoul, Korea, Republic of, 3Asan medical center, Seoul, Korea, Republic of.
Background: Pleuroparenchymal fibroelastosis (PPFE) is one of rare idiopathic interstitial pneumonias (IIP); however, clinical features and prognosis of Korean patients are not well defined.
Methods: A total of 26 patients, who were confirmed by lung biopsy (surgical lung biopsy: 15, transbronchial lung biopsy: 11) from 2010 to 2017 at Asan Medical Center, Seoul, Korea, were included and clinical data were retrospectively analyzed.
Results: Median follow-up period was 13.5 months. Mean age was 62.5±9.9 years old, 61.5% were men and 55% were smokers. Cough (69.2%) and dyspnea (80.8%) were the most frequent presenting symptoms. About half of the subjects had a history of pulmonary infection (42.3%), chemotherapy (11.5%) and organ transplantation (7.7%). Most patients (73.1%) showed restrictive pattern on lung function test (forced vital caapcity: 61.8±19.5% predicted, diffusing capacity of the lung for carbon monoxide: 55.3±18.4% predicted) and neutrophilia (14.5±17.7%) on bronchoalveolar larvage. In 65.4% of subjects, other interstitial lung disease (ILD) was also found in their lower lobes on chest computed tomography (CT) scan images, and in patients whose lower lobe was biopsied (n=15), usual interstitial pneumonia (UIP) pattern was the most common (40%) and fibroelastosis was found in 33.3%. Pneumothorax was developed in 26.9%. Eighteen (69.2%) patients were treated with steroid±immunosuppresants, but 15.4% died during follow-up (1-year survival rate: 89.2%, 2-year: 82.8%), mostly due to respiratory infection (75%). Subjects with lower-lobe involvement on chest CT scan images, showed older age (65.8±20.7 vs 56.3±18.9; p=0.066), lower residual volume (62.9±19.2 vs. 82.7±12.1; p=0.025) and shorter distance during six minute walk test (411.7±94.0 vs. 513.8±95.4; p=0.009), compared to those without; however, survival was similar (median survival period: seventeen months vs. twenty months; p=0.978 ).
Conclusions: About half of Korean PPFE patients had a relevant exposure history and two thirds of them had different ILD in their lower lobes, UIP pattern being the most common. Pneumothorax was common and survival was similar to other IIPs.
Key word : Pleuroparenchymal fibroelastosis, clinical features, prognosis
Table 1. Comparison of baseline characteristics between PPFE subjects with and without lower-lobe involvement.
Data are presented as No. (%) unless indicated otherwise
a BMI, body mass index; b FVC, forced vital capacity; FEV1, forced expiratory volume 1 second; DLco, diffusing capacity of the lungs for carbon monoxide; TLC, total lung capacity; RV, residual volume
c RVSP, right ventricular systolic pressure; d BAL, bronchoalveolar lavage
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