BACKGROUND

The primary aim of our study was to derive disease-specific outcomes following lung transplantation (LT) in patients with lymphangioleiomyomatosis (LAM).

METHODS

We queried the Organ Procurement and Transplant Network database to identify LAM patients that have undergone LT in the United States. The overall survival was analyzed with Kaplan–Meier curves. Survival estimates between subgroups of interest were compared using the log-rank method. Cox proportional hazard models were employed to determine the pre-transplant variables that impact post-LT survival.

RESULTS

One hundred and thirty-eight women with LAM underwent LT at 31 centers between January 2003 and June 2017. The median age at listing and transplant was 44 (IQR: 36-51) and 45 (IQR: 38-52) years, respectively. The median time spent on the LT waitlist was 257 (IQR: 85-616) days. The majority of the patients (109/134, 81%) received bilateral sequential LT. The median ischemic time was 4.9 (IQR: 4.1-6.1) hours. The actuarial Kaplan–Meier survival following LT for LAM patients at 1-, 5-, and 10 years was 94%, 73% and 56%, respectively. The post-LT survival was significantly better in LAM than in other lung diseases (10-year survival 56% vs. 32%, p < 0.01), and this advantage persisted after age- and gender-matched analysis (10-year survival 54% vs. 37%, p < 0.01). Pre-transplant parameters, such as the presence of pulmonary hypertension, six-minute walk distance, age at transplant, ischemic time during transplant, or type of transplant (single vs bilateral sequential LT), did not affect post-transplant survival.

CONCLUSIONS

The median survival after LT in LAM is 12 years and is substantially better than in other lung diseases.

中文翻译：

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淋巴管平滑肌瘤病患者肺移植后的临床结局和生存率。

背景

我们研究的主要目的是在患有肺血管平滑肌瘤病（LAM）的患者进行肺移植（LT）后得出特定疾病的结果。

方法

我们查询了器官采购和移植网络数据库，以识别在美国接受过LT治疗的LAM患者。使用Kaplan–Meier曲线分析了总生存期。使用对数秩方法比较了感兴趣的亚组之间的生存率估计值。使用Cox比例风险模型确定影响LT存活后的移植前变量。

结果

2003年1月至2017年6月，在31个中心对138名患有LAM的妇女进行了LT。上市和移植的中位年龄分别为44岁（IQR：36-51）和45岁（IQR：38-52）。在LT等待列表上花费的中位数时间为257天（IQR：85-616）天。大多数患者（109/134，81％）接受了双侧序贯性LT。中位数缺血时间为4.9（IQR：4.1-6.1）小时。LAM患者1年，5年和10年LT后的精算Kaplan–Meier存活率分别为94％，73％和56％。LAM患者的LT后生存率明显优于其他肺部疾病（10年生存率56％比32％，p <0.01），并且这一优势在经过年龄和性别匹配的分析后仍然存在（10年生存率54） ％对37％，p<0.01）。移植前的参数，例如是否存在肺动脉高压，六分钟的步行距离，移植时的年龄，移植过程中的缺血时间或移植类型（单次或双侧序贯LT），均不会影响移植后的生存。

结论

LAM中LT后的中位生存期为12年，明显好于其他肺部疾病。