Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study
Abstract Background Radiological pleuroparenchymal fibroelastosis (PPFE) lesion is characterized by pleural thickening with associated signs of subpleural fibrosis on high-resolution computed tomography (HRCT). This study evaluated the clinical significance of radiological PPFE as an isolated findin...
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2018-08-01
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Online Access: | http://link.springer.com/article/10.1186/s12931-018-0860-6 |
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English |
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Article |
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DOAJ |
author |
Kiminobu Tanizawa Tomohiro Handa Takeshi Kubo Toyofumi F. Chen-Yoshikawa Akihiro Aoyama Hideki Motoyama Kyoko Hijiya Akihiko Yoshizawa Yohei Oshima Kohei Ikezoe Shinsaku Tokuda Yoshinari Nakatsuka Yuko Murase Sonoko Nagai Shigeo Muro Toru Oga Kazuo Chin Toyohiro Hirai Hiroshi Date |
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Kiminobu Tanizawa Tomohiro Handa Takeshi Kubo Toyofumi F. Chen-Yoshikawa Akihiro Aoyama Hideki Motoyama Kyoko Hijiya Akihiko Yoshizawa Yohei Oshima Kohei Ikezoe Shinsaku Tokuda Yoshinari Nakatsuka Yuko Murase Sonoko Nagai Shigeo Muro Toru Oga Kazuo Chin Toyohiro Hirai Hiroshi Date Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study Respiratory Research Pleuroparenchymal fibroelastosis Lung transplantation Interstitial lung disease Idiopathic pulmonary fibrosis Survival |
author_facet |
Kiminobu Tanizawa Tomohiro Handa Takeshi Kubo Toyofumi F. Chen-Yoshikawa Akihiro Aoyama Hideki Motoyama Kyoko Hijiya Akihiko Yoshizawa Yohei Oshima Kohei Ikezoe Shinsaku Tokuda Yoshinari Nakatsuka Yuko Murase Sonoko Nagai Shigeo Muro Toru Oga Kazuo Chin Toyohiro Hirai Hiroshi Date |
author_sort |
Kiminobu Tanizawa |
title |
Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study |
title_short |
Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study |
title_full |
Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study |
title_fullStr |
Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study |
title_full_unstemmed |
Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study |
title_sort |
clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study |
publisher |
BMC |
series |
Respiratory Research |
issn |
1465-993X |
publishDate |
2018-08-01 |
description |
Abstract Background Radiological pleuroparenchymal fibroelastosis (PPFE) lesion is characterized by pleural thickening with associated signs of subpleural fibrosis on high-resolution computed tomography (HRCT). This study evaluated the clinical significance of radiological PPFE as an isolated finding or associated with other interstitial lung diseases (ILDs) in patients having fibrotic ILDs and registered for cadaveric lung transplantation (LT). Methods This retrospective study included 118 fibrotic ILD patients registered for LT. Radiological PPFE on HRCT was assessed. The impact of radiological PPFE on clinical features and transplantation-censored survival were evaluated. Results Radiological PPFE was observed in 30/118 cases (25%): definite PPFE (PPFE concentrated in the upper lobes, with involvement of lower lobes being less marked) in 12 (10%) and consistent PPFE (PPFE not concentrated in the upper lobes, or PPFE with features of coexistent disease present elsewhere) in 18 (15%). Of these, 12 had late-onset non-infectious pulmonary complications after hematopoietic stem-cell transplantation and/or chemotherapy (LONIPCs), 9 idiopathic PPFE, and 9 other fibrotic ILDs (idiopathic pulmonary fibrosis, IPF; other idiopathic interstitial pneumonias, other IIPs; connective tissue disease-associated ILD, CTD-ILD, and hypersensitivity pneumonia, HP). Radiological PPFE was associated with previous history of pneumothorax, lower body mass index, lower percentage of predicted forced vital capacity (%FVC), higher percentage of predicted diffusion capacity of carbon monoxide, less desaturation on six-minute walk test, and hypercapnia. The median survival time of all study cases was 449 days. Thirty-seven (28%) received LTs: cadaveric in 31 and living-donor lobar in six. Of 93 patients who did not receive LT, 66 (71%) died. Radiological PPFE was marginally associated with better survival after adjustment for age, sex, %FVC, and six-minute walk distance < 250 m (hazard ratio 0.51 [0.25–1.05], p = 0.07). After adjustment for covariates, idiopathic PPFE and LONIPC with radiological PPFE was associated with better survival than fibrotic ILDs without radiological PPFE (hazard ratio 0.38 [0.16–0.90], p = 0.03), and marginally better survival than other fibrotic ILDs with radiological PPFE (hazard ratio, 0.20 [0.04–1.11], p = 0.07). Conclusions idiopathic PPFE and LONIPC with radiological PPFE has better survival on the wait list for LT than fibrotic ILDs without radiological PPFE, after adjustment for age, sex, %FVC, and six-minute walk distance. |
topic |
Pleuroparenchymal fibroelastosis Lung transplantation Interstitial lung disease Idiopathic pulmonary fibrosis Survival |
url |
http://link.springer.com/article/10.1186/s12931-018-0860-6 |
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doaj-e5dcbc2a463547979051ae1438e837132020-11-25T00:40:41ZengBMCRespiratory Research1465-993X2018-08-0119111110.1186/s12931-018-0860-6Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort studyKiminobu Tanizawa0Tomohiro Handa1Takeshi Kubo2Toyofumi F. Chen-Yoshikawa3Akihiro Aoyama4Hideki Motoyama5Kyoko Hijiya6Akihiko Yoshizawa7Yohei Oshima8Kohei Ikezoe9Shinsaku Tokuda10Yoshinari Nakatsuka11Yuko Murase12Sonoko Nagai13Shigeo Muro14Toru Oga15Kazuo Chin16Toyohiro Hirai17Hiroshi Date18Department of Respiratory Care and Sleep Control Medicine, Graduate School of Medicine, Kyoto UniversityDepartment of Respiratory Medicine, Graduate School of Medicine, Kyoto UniversityDepartment of Diagnostic Imaging and Nuclear Medicine, Graduate School of Medicine, Kyoto UniversityDepartment of Thoracic Surgery, Graduate School of Medicine, Kyoto UniversityDepartment of Thoracic Surgery, Graduate School of Medicine, Kyoto UniversityDepartment of Thoracic Surgery, Graduate School of Medicine, Kyoto UniversityDepartment of Thoracic Surgery, Graduate School of Medicine, Kyoto UniversityDepartment of Diagnostic Pathology, Graduate School of Medicine, Kyoto UniversityDepartment of Rehabilitation, Kyoto University HospitalDepartment of Respiratory Medicine, Graduate School of Medicine, Kyoto UniversityDepartment of Respiratory Medicine, Graduate School of Medicine, Kyoto UniversityDepartment of Respiratory Medicine, Graduate School of Medicine, Kyoto UniversityDepartment of Respiratory Medicine, Graduate School of Medicine, Kyoto UniversityKyoto Central Clinic, Clinical Research CenterDepartment of Respiratory Medicine, Graduate School of Medicine, Kyoto UniversityDepartment of Respiratory Care and Sleep Control Medicine, Graduate School of Medicine, Kyoto UniversityDepartment of Respiratory Care and Sleep Control Medicine, Graduate School of Medicine, Kyoto UniversityDepartment of Respiratory Medicine, Graduate School of Medicine, Kyoto UniversityDepartment of Thoracic Surgery, Graduate School of Medicine, Kyoto UniversityAbstract Background Radiological pleuroparenchymal fibroelastosis (PPFE) lesion is characterized by pleural thickening with associated signs of subpleural fibrosis on high-resolution computed tomography (HRCT). This study evaluated the clinical significance of radiological PPFE as an isolated finding or associated with other interstitial lung diseases (ILDs) in patients having fibrotic ILDs and registered for cadaveric lung transplantation (LT). Methods This retrospective study included 118 fibrotic ILD patients registered for LT. Radiological PPFE on HRCT was assessed. The impact of radiological PPFE on clinical features and transplantation-censored survival were evaluated. Results Radiological PPFE was observed in 30/118 cases (25%): definite PPFE (PPFE concentrated in the upper lobes, with involvement of lower lobes being less marked) in 12 (10%) and consistent PPFE (PPFE not concentrated in the upper lobes, or PPFE with features of coexistent disease present elsewhere) in 18 (15%). Of these, 12 had late-onset non-infectious pulmonary complications after hematopoietic stem-cell transplantation and/or chemotherapy (LONIPCs), 9 idiopathic PPFE, and 9 other fibrotic ILDs (idiopathic pulmonary fibrosis, IPF; other idiopathic interstitial pneumonias, other IIPs; connective tissue disease-associated ILD, CTD-ILD, and hypersensitivity pneumonia, HP). Radiological PPFE was associated with previous history of pneumothorax, lower body mass index, lower percentage of predicted forced vital capacity (%FVC), higher percentage of predicted diffusion capacity of carbon monoxide, less desaturation on six-minute walk test, and hypercapnia. The median survival time of all study cases was 449 days. Thirty-seven (28%) received LTs: cadaveric in 31 and living-donor lobar in six. Of 93 patients who did not receive LT, 66 (71%) died. Radiological PPFE was marginally associated with better survival after adjustment for age, sex, %FVC, and six-minute walk distance < 250 m (hazard ratio 0.51 [0.25–1.05], p = 0.07). After adjustment for covariates, idiopathic PPFE and LONIPC with radiological PPFE was associated with better survival than fibrotic ILDs without radiological PPFE (hazard ratio 0.38 [0.16–0.90], p = 0.03), and marginally better survival than other fibrotic ILDs with radiological PPFE (hazard ratio, 0.20 [0.04–1.11], p = 0.07). Conclusions idiopathic PPFE and LONIPC with radiological PPFE has better survival on the wait list for LT than fibrotic ILDs without radiological PPFE, after adjustment for age, sex, %FVC, and six-minute walk distance.http://link.springer.com/article/10.1186/s12931-018-0860-6Pleuroparenchymal fibroelastosisLung transplantationInterstitial lung diseaseIdiopathic pulmonary fibrosisSurvival |