BACKGROUND Pleuroparenchymal fibroelastosis (PPFE) has no currently available specific treatment. Benefits of lung transplantation (LT) for PPFE are poorly documented. METHODS We conducted a nation-wide multicentric retrospective study in patients who underwent lung or heart-lung transplantation for chronic end-stage lung disease secondary to PPFE between 2012 and 2022 in France. RESULTS Thirty-one patients were included. At transplantation, median age was 48 years [IQR 35-55]. About 64.5% were women. Twenty-one (67.7%) had idiopathic PFFE. Sixteen (52%) had bilateral LT, 10 (32%) had single LT, 4 (13%) had lobar transplantation and one (3%) had heart-lung transplantation. Operative mortality was 3.2%. Early mortality (<90 days or during the first hospitalization) was 32%. Eleven patients (35.5%) underwent reoperation for hemostasis. Eight (30.8%) experienced bronchial complications. Mechanical ventilation time was 10 days [IQR 2-55]. Length of stay in intensive care unit and hospital were 34 [IQR 18-73] and 64 [IQR 36-103] days, respectively. Median survival was 21 months. Post-transplant survival rates after 1, 2, and 5 years were 57.9%, 42.6% and 38.3% respectively. Low albuminemia (p = 0.046), FVC (p = 0.021), FEV1 (p = 0.009) and high emergency lung transplantation (p = 0.04) were associated with increased early mortality. Oversized graft tended to be correlated to a higher mortality (p = 0.07). CONCLUSION LT for PPFE is associated with high post-operative morbi-mortality rates. Patients requiring high emergency lung transplantation with advanced disease, malnutrition, or critical clinical status experienced worse outcomes. CLINICALTRIALS GOV IDENTIFIER NCT05044390.

中文翻译：

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肺移植治疗胸膜实质弹力纤维增生症的结果：法国多中心回顾性研究。


背景 胸膜实质弹力纤维增生症（PPFE）目前没有可用的特异性治疗方法。肺移植 (LT) 对 PPFE 的益处鲜有记录。方法 我们对 2012 年至 2022 年间在法国因 PPFE 继发慢性终末期肺病接受肺或心肺移植的患者进行了一项全国范围的多中心回顾性研究。结果 纳入 31 名患者。移植时，中位年龄为 48 岁 [IQR 35-55]。大约 64.5% 是女性。 21 人 (67.7%) 患有特发性 PFFE。 16 例（52%）接受双侧 LT，10 例（32%）接受单侧 LT，4 例（13%）接受肺叶移植，1 例（3%）接受心肺移植。手术死亡率为3.2%。早期死亡率（<90 天或首次住院期间）为 32%。 11名患者（35.5%）因止血而再次手术。八名 (30.8%) 出现支气管并发症。机械通气时间为 10 天 [IQR 2-55]。重症监护室和医院的住院时间分别为 34 天 [IQR 18-73] 和 64 天 [IQR 36-103] 天。中位生存期为 21 个月。移植后1年、2年和5年的存活率分别为57.9%、42.6%和38.3%。低白蛋白血症（p = 0.046）、FVC（p = 0.021）、FEV1（p = 0.009）和高紧急肺移植（p = 0.04）与早期死亡率增加相关。过大的移植物往往与较高的死亡率相关（p = 0.07）。结论 PPFE 的 LT 与高术后死亡率相关。患有晚期疾病、营养不良或临床状态危急、需要高度紧急肺移植的患者预后较差。临床试验政府标识符 NCT05044390。