Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up
Background/Aims Pleuroparenchymal fibroelastosis (PPFE) is a type of rare idiopathic interstitial pneumonia that is characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal fibrosis. This study aimed to determine the clinico-radiologic-pathologic features...
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| Format: | Article |
| Language: | English |
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The Korean Association of Internal Medicine
2021-03-01
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| Series: | The Korean Journal of Internal Medicine |
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| Online Access: | http://www.kjim.org/upload/pdf/kjim-2019-303.pdf |
| _version_ | 1819146048385318912 |
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| author | Jae Ha Lee Eun Jin Chae Joon Seon Song Miae Kim Jin Woo Song |
| author_facet | Jae Ha Lee Eun Jin Chae Joon Seon Song Miae Kim Jin Woo Song |
| author_sort | Jae Ha Lee |
| collection | DOAJ |
| description | Background/Aims Pleuroparenchymal fibroelastosis (PPFE) is a type of rare idiopathic interstitial pneumonia that is characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal fibrosis. This study aimed to determine the clinico-radiologic-pathologic features and prognosis of Korean patients with PPFE. Methods A total of 26 patients who were confirmed to have PPFE by lung biopsy, were included, and their clinico-radiologic-pathologic findings were retrospectively analyzed. Results The mean follow-up period was 23.8 months, and the mean age of the patients was 62.5 years; 61.5% were men and 50% were smokers. Cough and dyspnea were the most frequent presenting symptoms, and restrictive pattern was the most common observation in lung function. In 84.6% of the subjects, lower lobe involvement was found on chest computed tomography, and the usual interstitial pneumonia (UIP) pattern was the most common (59.1%). Among patients whose lower lobe was biopsied (n = 13), the UIP pattern was the most common (46.2%). Patients with lower lobe involvement were older and walked a shorter distance during the 6-minute walk test, compared to those without. Spontaneous pneumothorax was a common complication (26.9%), and 15.4% of the patients died mostly due to pneumonia (100%). The 1- and 3-year survival rates were 90.2% and 84.5%, respectively. Conclusions Clinical features of Korean patients with PPFE were similar to those reported previously; however, lower lobe involvement was more frequent. During follow-up, one-fourth of the patients experienced pneumothorax and one-fifth died from pneumonia. |
| first_indexed | 2024-12-22T13:07:44Z |
| format | Article |
| id | doaj.art-8a4878cb2b5b41afb6a91227675981cb |
| institution | Directory Open Access Journal |
| issn | 1226-3303 2005-6648 |
| language | English |
| last_indexed | 2024-12-22T13:07:44Z |
| publishDate | 2021-03-01 |
| publisher | The Korean Association of Internal Medicine |
| record_format | Article |
| series | The Korean Journal of Internal Medicine |
| spelling | doaj.art-8a4878cb2b5b41afb6a91227675981cb2022-12-21T18:24:49ZengThe Korean Association of Internal MedicineThe Korean Journal of Internal Medicine1226-33032005-66482021-03-0136Suppl 1S132S14110.3904/kjim.2019.303170312Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-upJae Ha Lee0Eun Jin Chae1Joon Seon Song2Miae Kim3Jin Woo Song4 Division of Pulmonology and Critical Care Medicine, Department of Internal Medicine, Inje niversity Haeundae Paik Hospital, Busan, Korea Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, KoreaBackground/Aims Pleuroparenchymal fibroelastosis (PPFE) is a type of rare idiopathic interstitial pneumonia that is characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal fibrosis. This study aimed to determine the clinico-radiologic-pathologic features and prognosis of Korean patients with PPFE. Methods A total of 26 patients who were confirmed to have PPFE by lung biopsy, were included, and their clinico-radiologic-pathologic findings were retrospectively analyzed. Results The mean follow-up period was 23.8 months, and the mean age of the patients was 62.5 years; 61.5% were men and 50% were smokers. Cough and dyspnea were the most frequent presenting symptoms, and restrictive pattern was the most common observation in lung function. In 84.6% of the subjects, lower lobe involvement was found on chest computed tomography, and the usual interstitial pneumonia (UIP) pattern was the most common (59.1%). Among patients whose lower lobe was biopsied (n = 13), the UIP pattern was the most common (46.2%). Patients with lower lobe involvement were older and walked a shorter distance during the 6-minute walk test, compared to those without. Spontaneous pneumothorax was a common complication (26.9%), and 15.4% of the patients died mostly due to pneumonia (100%). The 1- and 3-year survival rates were 90.2% and 84.5%, respectively. Conclusions Clinical features of Korean patients with PPFE were similar to those reported previously; however, lower lobe involvement was more frequent. During follow-up, one-fourth of the patients experienced pneumothorax and one-fifth died from pneumonia.http://www.kjim.org/upload/pdf/kjim-2019-303.pdfidiopathic interstitial pneumoniaspulmonary fibrosispleural diseasessurvival analysis |
| spellingShingle | Jae Ha Lee Eun Jin Chae Joon Seon Song Miae Kim Jin Woo Song Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up The Korean Journal of Internal Medicine idiopathic interstitial pneumonias pulmonary fibrosis pleural diseases survival analysis |
| title | Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up |
| title_full | Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up |
| title_fullStr | Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up |
| title_full_unstemmed | Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up |
| title_short | Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up |
| title_sort | pleuroparenchymal fibroelastosis in korean patients clinico radiologic pathologic features and 2 year follow up |
| topic | idiopathic interstitial pneumonias pulmonary fibrosis pleural diseases survival analysis |
| url | http://www.kjim.org/upload/pdf/kjim-2019-303.pdf |
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