Outcomes of lung transplantation for idiopathic pleuroparenchymal fibroelastosis
Outcomes of lung transplantation for idiopathic pleuroparenchymal fibroelastosis
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DOI：
10.1007/s00595-021-02232-6
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发表时间：
2021-02
期刊：
影响因子：
2.5
通讯作者：
H. Shiiya;J. Nakajima;H. Date;T. Chen-Yoshikawa;K. Tanizawa;T. Handa;T. Oto;S. Otani;T. Shiotani;Y. Okada;Y. Matsuda;T. Shiraishi;T. Moroga;M. Minami;S. Funaki;M. Chida;I. Yoshino;G. Hatachi;Y. Uemura;M. Sato
中科院分区：
文献类型：
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作者：
H. Shiiya;J. Nakajima;H. Date;T. Chen-Yoshikawa;K. Tanizawa;T. Handa;T. Oto;S. Otani;T. Shiotani;Y. Okada;Y. Matsuda;T. Shiraishi;T. Moroga;M. Minami;S. Funaki;M. Chida;I. Yoshino;G. Hatachi;Y. Uemura;M. Sato
目的本研究旨在比较肺移植（LT）治疗特发性胸膜实质纤维弹性组织增生症（IPPFE）和LT治疗特发性肺纤维化（IPF）的结局。方法我们回顾性分析了1998年至2018年期间在日本接受LT治疗IPPFE或IPF的所有成人患者。IPPFE患者的体重指数（BMI）往往显著低于IPF患者（中位数分别为16.7和22.6 kg/m2;P< 0.01）。然而，Kaplan-Meier生存曲线显示两组间的总生存期无显著差异。IPPFE患者的BMI没有增加，甚至在LT后1年（移植前，16.5 ± 3.2 kg/m2 vs.移植后1年，15.6 ± 2.5 kg/m2;P= 0.08）。IPPFE组LT后1年的预测用力肺活量百分比（%FVC）显著低于IPF组（分别为48.4% ± 19.5%和68.6% ± 15.5%;结论尽管IPPFE患者存在肺外问题，如平坦胸部、低BMI和相关的限制性功能障碍，IPPFE或IPF LT后的患者生存率相当。
PurposeThis 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).MethodsWe reviewed, retrospectively, all adult patients who underwent LT for IPPFE or IPF in Japan between 1998 and 2018.ResultsThere 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/m2vs. 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).ConclusionsDespite 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.