Pulmonary Division, Heart Institute (InCor), University of São Paulo Medical School, Av Dr Enéas de Carvalho Aguiar, 44, 5º andar - sala 1, São Paulo, 05403-900, Brazil.
Thoracic Surgery Division, Heart Institute (InCor), University of São Paulo Medical School, São Paulo, Brazil.
Lung. 2017 Dec;195(6):699-705. doi: 10.1007/s00408-017-0045-y. Epub 2017 Aug 19.
Lung transplantation (LT) is the standard of care for patients with advanced lung diseases, including lymphangioleiomyomatosis (LAM). LAM accounts for only 1% of all LTs performed in the international registry. As a result, the global experience, including the use of mechanistic target of rapamycin (mTOR) inhibitors before and after LT in LAM, is still limited.
We conducted a retrospective review of all LAM patients who underwent LT at our centre between 2003 and 2016. Pre- and post-transplant data were assessed.
Eleven women with LAM underwent LT, representing 3.3% of all procedures. Ten (91%) patients underwent double-LT. The mean age at diagnosis was 39 ± 6 years and the mean FEV before LT was 28 ± 14%. Only one patient underwent pleurodesis for recurrent pneumothorax. Pulmonary hypertension was confirmed in 3 (27%) patients. Four (36%) patients received sirolimus preoperatively; three of them received it until the day of LT, and there was no occurrence of bronchial anastomotic dehiscence after the procedure. Four patients (36%) received mTOR inhibitors post-transplant. The median follow-up from LT was 44 months. There were 3 deaths (27%) during the study and survival probabilities at 1, 3, and 5 years after LT were, 90, 90, and 77%, respectively.
This data reinforces the role of LT for LAM patients with end-stage disease. The use of sirolimus seems to be safe before LT and the occurrence of complications after LT, including those LAM-related, should be continuously monitored.
肺移植（LT）是治疗晚期肺部疾病患者的标准治疗方法，包括淋巴管平滑肌瘤病（LAM）。LAM 仅占国际登记处所有 LT 的 1%。因此，包括在 LT 前后使用雷帕霉素（mTOR）抑制剂在内的全球经验仍然有限。
我们对 2003 年至 2016 年期间在我们中心接受 LT 的所有 LAM 患者进行了回顾性研究。评估了移植前后的数据。
11 名患有 LAM 的女性接受了 LT，占所有手术的 3.3%。十名（91%）患者接受了双肺 LT。诊断时的平均年龄为 39 ± 6 岁，LT 前的平均 FEV 为 28 ± 14%。只有一名患者因复发性气胸而行胸膜固定术。3 名（27%）患者确诊为肺动脉高压。4 名（36%）患者术前接受了西罗莫司治疗；其中 3 名患者在 LT 当天接受了治疗，术后未发生支气管吻合口裂开。4 名（36%）患者在移植后接受了 mTOR 抑制剂治疗。从 LT 开始的中位随访时间为 44 个月。研究期间有 3 例死亡（27%），LT 后 1、3 和 5 年的生存率分别为 90%、90%和 77%。
这些数据强化了 LT 对晚期 LAM 患者的作用。在 LT 之前使用西罗莫司似乎是安全的，并且应该持续监测 LT 后发生的并发症，包括与 LAM 相关的并发症。
