Haruhiko Shiiya1,2, Jun Nakajima1, Hiroshi Date3, Toyofumi Fengshi Chen-Yoshikawa3,4, Kiminobu Tanizawa5, Tomohiro Handa6, Takahiro Oto7, Shinji Otani7, Toshio Shiotani7, Yoshinori Okada8, Yasushi Matsuda8, Takeshi Shiraishi9, Toshihiko Moroga9, Masato Minami10, Soichiro Funaki10, Masayuki Chida11, Ichiro Yoshino12, Go Hatachi13, Yukari Uemura14, Masaaki Sato15. 1. Department of Thoracic Surgery, The University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. 2. Department of Cardiovascular and Thoracic Surgery, Hokkaido University Graduate School of Medicine, Hokkaido, Japan. 3. Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan. 4. Department of Thoracic Surgery, Nagoya University Graduate School of Medicine, Nagoya, Japan. 5. Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan. 6. Department of Advanced Medicine for Respiratory Failure, Graduate School of Medicine, Kyoto University, Kyoto, Japan. 7. Department of Organ Transplant Center/General Thoracic Surgery, Okayama University Hospital, Okayama, Japan. 8. Department of Thoracic Surgery, Institute of Development, Aging and Cancer, Tohoku University, Miyagi, Japan. 9. Department of General Thoracic, Breast and Pediatric Surgery, Fukuoka University School of Medicine, Fukuoka, Japan. 10. Department of General Thoracic Surgery, Osaka University Graduate School of Medicine, Osaka, Japan. 11. Department of General Thoracic Surgery, Dokkyo Medical University, Tochigi, Japan. 12. Department of General Thoracic Surgery, Chiba University Graduate School of Medicine, Chiba, Japan. 13. Department of Surgical Oncology, Nagasaki University Graduate School of Medicine, Nagasaki, Japan. 14. Biostatistics Section, Department of Data Science, Center for Clinical Sciences, National Center for Global Health and Medicine, Tokyo, Japan. 15. Department of Thoracic Surgery, The University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. satom-sur@h.u-tokyo.ac.jp.
Abstract
PURPOSE: This study was performed to compare the outcome of lung transplantation (LT) for idiopathic pleuroparenchymal fibroelastosis (IPPFE) with that of LT for idiopathic pulmonary fibrosis (IPF). METHODS: We reviewed, retrospectively, all adult patients who underwent LT for IPPFE or IPF in Japan between 1998 and 2018. RESULTS: There were 100 patients eligible for this study (31 with IPPFE and 69 with IPF). Patients with IPPFE tended to have a significantly lower body mass index (BMI) than those with IPF (median, 16.7 vs. 22.6 kg/m2, respectively; P < 0.01). However, Kaplan-Meier survival curves showed no significant difference in overall survival between the groups. The BMI did not increase in patients with IPPFE, even 1 year after LT (pretransplant, 16.5 ± 3.2 kg/m2 vs. 1 year post-transplant, 15.6 ± 2.5 kg/m2; P = 0.08). The percent predicted forced vital capacity (%FVC) 1 year after LT was significantly lower in the IPPFE group than in the IPF group (48.4% ± 19.5% vs. 68.6% ± 15.5%, respectively; P < 0.01). CONCLUSIONS: Despite extrapulmonary problems such as a flat chest, low BMI, and associated restrictive impairment persisting in patients with IPPFE, patient survival after LT for IPPFE or IPF was equivalent.
PURPOSE: This study was performed to compare the outcome of lung transplantation (LT) for idiopathic pleuroparenchymal fibroelastosis (IPPFE) with that of LT for idiopathic pulmonary fibrosis (IPF). METHODS: We reviewed, retrospectively, all adult patients who underwent LT for IPPFE or IPF in Japan between 1998 and 2018. RESULTS: There were 100 patients eligible for this study (31 with IPPFE and 69 with IPF). Patients with IPPFE tended to have a significantly lower body mass index (BMI) than those with IPF (median, 16.7 vs. 22.6 kg/m2, respectively; P < 0.01). However, Kaplan-Meier survival curves showed no significant difference in overall survival between the groups. The BMI did not increase in patients with IPPFE, even 1 year after LT (pretransplant, 16.5 ± 3.2 kg/m2 vs. 1 year post-transplant, 15.6 ± 2.5 kg/m2; P = 0.08). The percent predicted forced vital capacity (%FVC) 1 year after LT was significantly lower in the IPPFE group than in the IPF group (48.4% ± 19.5% vs. 68.6% ± 15.5%, respectively; P < 0.01). CONCLUSIONS: Despite extrapulmonary problems such as a flat chest, low BMI, and associated restrictive impairment persisting in patients with IPPFE, patient survival after LT for IPPFE or IPF was equivalent.
Authors: Merih Oray; Khawla Abu Samra; Nazanin Ebrahimiadib; Halea Meese; C Stephen Foster Journal: Expert Opin Drug Saf Date: 2016-02-06 Impact factor: 4.250