Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up
2
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
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.
Abstract
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.
참고문헌 (33)
- Travis WD, "An official American Thoracic Society", European Respiratory Society statement : update of the international multidisciplinary classification of the idiopathic interstitial pneumonias, 2013, pp.188, 733~748
- Frankel SK, "Idiopathic pleuroparenchymal fibroelastosis : description of a novel clinicopathologic entity", Chest, 126, pp.2007~2013 (2004)
- Reddy TL, "Pleuroparen chymal fibroelastosis : a spectrum of histopathological and imaging phenotypes", Eur Respir J, 40, pp.377~385 (2012)
- Nakatani T, "Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity?", Eur Respir J, 45, pp.1183~1186 (2015)
- Watanabe K, "Rapid decrease in forced vital capacity in patients with idiopathic pulmonary upper lobe fibrosis", Respir Investig, 50, pp.88~97 (2012)
- Watanabe K, "Pleuroparenchymal fibroelastosis : its clinical characteristics", Curr Respir Med Rev, 9, pp.299~237 (2013)
- von der Thusen JH, "Pleuroparenchymal fibroelastosis : its pathological characteristics", Curr Respir Med Rev, 9, pp.238~247 (2013)
- Yoshida Y, "Heterogeneous clinical features in patients with pulmonary fibrosis showing histology of pleuroparenchymal fibroelastosis", Respir Investig, 54, pp.162~169 (2016)
- Enomoto Y, "Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis : a retrospective multicenter study", Respir Med, 133, pp.1~5 (2017)
- Park CS, "Nonspecific interstitial pneumonia", fibrosis : clinical manifestations, histologic and radiologic features, 1996, pp.11, 122~132
- Lim GI, "Clinical features of interstitial lung diseases", Korean J Intern Med, 11, pp.113~121 (1996)
- Cha YJ, "Pleuroparenchymal fibroelastosis in heterogeneous clinical conditions : clinicopathologic analysis of 7 cases", Clin Respir J, 12, pp.1495~1502 (2018)
- Wanger J, "Standardisation of the measurement of lung volumes", Eur Respir J, 26, pp.511~522 (2005)
- Miller MR, "Standardisation of spirometry", Eur Respir J, 26, pp.319~338 (2005)
- Macintyre N, "Standardisation of the single-breath determination of carbon monoxide uptake in the lung", Eur Respir J, 26, pp.720~735 (2005)
- Oda T, "Distinct characteristics of pleuroparenchymal fibroelastosis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis", Chest, 146, pp.1248~1255 (2014)
- Ishii H, "Pleuroparenchymal fibroelastosis diagnosed by multidisciplinary discussions in Japan", Respir Med, 141, pp.190~197 (2018)
- Holland AE, "An official European Respiratory Society", American Thoracic Society technical standard : field walking tests in chronic respiratory disease, 2014, pp.44, 1428~1446
- Kato M, "Usual interstitial pneumonia pattern in the lower lung lobes as a prognostic factor in idiopathic pleuroparenchymal fibroelastosis", Respiration, 97, pp.319~328 (2019)
- Kusagaya H, "Idiopathic pleuroparenchymal fibroelastosis : consideration of a clinicopathological entity in a series of Japanese patients", BMC Pulm Med, 12, pp.72~ (2012)
- Tanizawa K, "Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation : a retrospective cohort study", Respir Res, 19, pp.162~ (2018)
- Suzuki Y, "Distinct profile and prognostic impact of body composition changes in idiopathic pulmonary fibrosis and idiopathic pleuroparenchymal fibroelastosis", Sci Rep, 8, pp.14074~ (2018)
- Mariani F, "Pleuroparenchymal fibroelastosis : the prevalence of secondary forms in hematopoietic stem cell and lung transplantation recipients", Diagn Interv Radiol, 22, pp.400~406 (2016)
- Takeuchi Y, "Pleuroparenchymal fibroelastosis and non-specific interstitial pneumonia : frequent pulmonary sequelae of haematopoietic stem cell transplantation", Histopathology, 66, pp.536~544 (2015)
- Ishii T, "Air-leak syndrome by pleuroparenchymal fibroelastosis after bone marrow transplantation", Intern Med, 55, pp.105~111 (2016)
- Kinoshita Y, "Pleuroparenchymal fibroelastosis as a histological background of autoimmune diseases", Virchows Arch, 474, pp.97~104 (2019)
- Esteves C, "Pleuroparenchymal fibroelastosis : role of high-resolution computed tomography(HRCT)and CT-guided transthoracic core lung biopsy", Insights Imaging, 7, pp.155~162 (2016)
- Kono M, "Clinical significance of lower-lobe interstitial lung disease on high-resolution computed tomography in patients with idiopathic pleuroparenchymal fibroelastosis", Respir Med, 154, pp.122~126 (2019)
- Enomoto N, "Quantitative analysis of lung elastic fibers in idiopathic pleuroparenchymal fibroelastosis(IPPFE) : comparison of clinical, radiological, and pathological findings with those of idiopathic pulmonary fibrosis(IPF)", BMC Pulm Med, 14, pp.91~ (2014)
- Shioya M, "Poorer prognosis of idiopathic pleuroparenchymal fibroelastosis compared with idiopathic pulmonary fibrosis in advanced stage", Can Respir J, 2018, pp.6043053~ (2018)
- Khiroya R, "Pleuroparenchymal fibroelastosis : a review of histopathologic features and the relationship between histologic parameters and survival", Am J Surg Pathol, 41, pp.1683~1689 (2017)
- Sato S, "A patient with idiopathic pleuroparenchymal fibroelastosis showing a sustained pulmonary function due to treatment with pirfenidone", Intern Med, 55, pp.497~501 (2016)
- Boerner EB, "Idiopathic pleuroparenchymal fibroelastosis (PPFE): a case study of a rare entity", Rev Port Pneumol(2006), 23, pp.352~355 (2017)