Outcomes of lung transplantation for idiopathic pleuroparenchymal fibroelastosis

Haruhiko Shiiya, Jun Nakajima, Hiroshi Date, Toyofumi Fengshi Chen-Yoshikawa, Kiminobu Tanizawa, Tomohiro Handa, Takahiro Oto, Shinji Otani, Toshio Shiotani, Yoshinori Okada, Yasushi Matsuda, Takeshi Shiraishi, Toshihiko Moroga, Masato Minami, Soichiro Funaki, Masayuki Chida, Ichiro Yoshino, Go Hatachi, Yukari Uemura, Masaaki Sato

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6 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)1276-1284
Number of pages9
JournalSurgery Today
Issue number8
Publication statusPublished - 2021 Aug


  • Chest wall
  • Interstitial lung disease
  • Lung
  • Pulmonary function
  • Survival analysis
  • Transplantation


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