Abstract：
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]. 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).
CONCLUSIONS: 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.
RESULTS:
UNASSIGNED: NCT05044390.
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]. 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).
CONCLUSIONS: 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.
RESULTS:
UNASSIGNED: NCT05044390.
摘要：
背景：胸膜实质纤维弹性增生症（PPFE）目前尚无可用的特异性治疗方法。肺移植（LT）对PPFE的益处很少有记载。
方法：我们在法国2012年至2022年期间因PPFE继发慢性终末期肺病而接受肺或心肺移植的患者中进行了一项全国范围的多中心回顾性研究。
结果：纳入31例患者。在移植时，中位年龄为48岁[IQR35-55]。64.5%是女性。21例(67.7%)患有特发性PFFE。16人（52%）有双边LT，10人(32%)有单一LT，4例（13％）进行了大叶移植，1例（3％）进行了心肺移植。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例(35.5%)患者接受了再次手术止血。8例（30.8%）出现支气管并发症。机械通气时间为10天[IQR2-55]。在重症监护室和医院的住院时间分别为34[IQR18-73]和64[IQR36-103]天,分别。中位生存期为21个月。移植后1年、2年和5年生存率分别为57.9%，分别为42.6%和38.3%。低白蛋白血症（p=0.046），FVC（p=0.021），FEV1（p=0.009）和高度紧急肺移植（p=0.04）与早期死亡率增加有关。过度的移植物倾向于与较高的死亡率相关(p=0.07)。
结论：LT治疗PPFE与高术后死亡率相关。需要高度紧急肺移植的晚期患者，营养不良,或危重的临床状态经历了更差的结果。
结果：
■NCT05044390。
方法：我们在法国2012年至2022年期间因PPFE继发慢性终末期肺病而接受肺或心肺移植的患者中进行了一项全国范围的多中心回顾性研究。
结果：纳入31例患者。在移植时，中位年龄为48岁[IQR35-55]。64.5%是女性。21例(67.7%)患有特发性PFFE。16人（52%）有双边LT，10人(32%)有单一LT，4例（13％）进行了大叶移植，1例（3％）进行了心肺移植。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例(35.5%)患者接受了再次手术止血。8例（30.8%）出现支气管并发症。机械通气时间为10天[IQR2-55]。在重症监护室和医院的住院时间分别为34[IQR18-73]和64[IQR36-103]天,分别。中位生存期为21个月。移植后1年、2年和5年生存率分别为57.9%，分别为42.6%和38.3%。低白蛋白血症（p=0.046），FVC（p=0.021），FEV1（p=0.009）和高度紧急肺移植（p=0.04）与早期死亡率增加有关。过度的移植物倾向于与较高的死亡率相关(p=0.07)。
结论：LT治疗PPFE与高术后死亡率相关。需要高度紧急肺移植的晚期患者，营养不良,或危重的临床状态经历了更差的结果。
结果：
■NCT05044390。
