Yuji Yoshida1, Nobuhiko Nagata2, Nobuko Tsuruta3, Yasuhiko Kitasato4, Kentaro Wakamatsu5, Michihiro Yoshimi6, Hiroshi Ishii7, Takako Hirota8, Naoki Hamada9, Masaki Fujita10, Kazuki Nabeshima11, Fumiaki Kiyomi12, Kentaro Watanabe13. 1. Department of Respiratory Medicine, Fukuoka University School of Medicine, Fukuoka 814-0180, Japan. Electronic address: sukehiko_yossan@yahoo.co.jp. 2. Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka 818-0067, Japan. Electronic address: nbnagata@fukuoka-u.ac.jp. 3. Department of Respiratory Medicine, Hamanomachi Hospital, Fukuoka 810-8539, Japan. Electronic address: tsuruta@hamanomachi.jp. 4. Department of Respiratory Medicine, Kurume General Hospital, Kurume 830-0013, Japan. Electronic address: kita-sir1028@jcom.home.ne.jp. 5. Department of Respiratory Medicine, National Hospital Organization, Omuta National Hospital, Omuta 837-0911, Japan. Electronic address: wakamatsu-k@oomuta-h.com. 6. Department of Respiratory Medicine, National Hospital Organization, Fukuoka Higashi Medical Center, Koga 811-3195, Japan. Electronic address: yoshimimi@fukuokae2.hosp.go.jp. 7. Department of Respiratory Medicine, Fukuoka University School of Medicine, Fukuoka 814-0180, Japan. Electronic address: hishii@fukuoka-u.ac.jp. 8. Department of Respiratory Medicine, Fukuoka University School of Medicine, Fukuoka 814-0180, Japan. Electronic address: tahirota@fukuoka-u.ac.jp. 9. Department of Respiratory Medicine, Kyushu University Hospital, Fukuoka 812-8582, Japan. Electronic address: n-hamada@kokyu.med.kyushu-u.ac.jp. 10. Department of Respiratory Medicine, Fukuoka University School of Medicine, Fukuoka 814-0180, Japan. Electronic address: mfujita@fukuoka-u.ac.jp. 11. Department of Pathology, Fukuoka University School of Medicine, Fukuoka 814-0180, Japan. Electronic address: kaznabes@fukuoka-u.ac.jp. 12. Academia, Industry, and Government Collaborative Research Institute of Translational Medicine for Life Innovation, Fukuoka University, Fukuoka 814-0180, Japan. Electronic address: fumkiyomi@adm.fukuoka-u.ac.jp. 13. Department of Respiratory Medicine, Fukuoka University School of Medicine, Fukuoka 814-0180, Japan. Electronic address: watanabe@fukuoka-u.ac.jp.
Abstract
BACKGROUND: The histological pattern of pleuroparenchymal fibroelastosis (PPFE) is well defined, but its clinical features remain unclear. METHODS: We retrospectively examined the predominantly involved lung-fields (based on abnormal opacities on computed tomography [CT] images), and the initial value and annual decline of respiratory function in patients with pulmonary fibrosis presenting with histologically confirmed PPFE. RESULTS: Thirteen female and nine male subjects were included. Eleven interpreters independently analyzed 231 CT image series. One-third of the CT series (78/231) was interpreted as demonstrating equal involvement of the upper and lower lung fields, i.e., six out of 21 patients had equal involvement of the upper and lower lung fields, based on a majority decision of the interpreters. The residual volume/total lung capacity (RV/TLC) was increased and correlated inversely with forced vital capacity (FVC) at the initial measurement. FVC followed two patterns of decline over time: a gradual decline over a follow-up period of more than 6 years (-55mL/year, R(2)=0.799), and a relatively rapid decline over a shorter period (-364mL/year, R(2)=0.855) as determined by mixed-effect linear regression. CONCLUSIONS: The predominantly involved sites seen on CT images of PPFE were not limited to the upper lobes. In some cases, upper lung fields were predominantly involved, but in other cases, both upper and lower lung fields were equally involved. Two patterns of FVC decline exists: a rapid decline over a short period and a slow decline over a longer period, suggesting that the disease follows a heterogeneous clinical course.
BACKGROUND: The histological pattern of pleuroparenchymal fibroelastosis (PPFE) is well defined, but its clinical features remain unclear. METHODS: We retrospectively examined the predominantly involved lung-fields (based on abnormal opacities on computed tomography [CT] images), and the initial value and annual decline of respiratory function in patients with pulmonary fibrosis presenting with histologically confirmed PPFE. RESULTS: Thirteen female and nine male subjects were included. Eleven interpreters independently analyzed 231 CT image series. One-third of the CT series (78/231) was interpreted as demonstrating equal involvement of the upper and lower lung fields, i.e., six out of 21 patients had equal involvement of the upper and lower lung fields, based on a majority decision of the interpreters. The residual volume/total lung capacity (RV/TLC) was increased and correlated inversely with forced vital capacity (FVC) at the initial measurement. FVC followed two patterns of decline over time: a gradual decline over a follow-up period of more than 6 years (-55mL/year, R(2)=0.799), and a relatively rapid decline over a shorter period (-364mL/year, R(2)=0.855) as determined by mixed-effect linear regression. CONCLUSIONS: The predominantly involved sites seen on CT images of PPFE were not limited to the upper lobes. In some cases, upper lung fields were predominantly involved, but in other cases, both upper and lower lung fields were equally involved. Two patterns of FVC decline exists: a rapid decline over a short period and a slow decline over a longer period, suggesting that the disease follows a heterogeneous clinical course.
Authors: Carlos A C Pereira; Maria R Soares; Rafaela Boaventura; Marina D C Castro; Paula S Gomes; Andrea Gimenez; Cesar Fukuda; Milena Cerezoli; Israel Missrie Journal: Medicine (Baltimore) Date: 2019-07 Impact factor: 1.817
Authors: Felix Chua; Sujal R Desai; Andrew G Nicholson; Anand Devaraj; Elisabetta Renzoni; Alexandra Rice; Athol U Wells Journal: Ann Am Thorac Soc Date: 2019-11