Department of Radiology, Ansan Hospital, Korea University College of Medicine, 123, Jeokgeum-ro, Danwon-gu, Ansan-si, Gyeonggi, 15355, South Korea.
Department of Radiology and the Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, 86 Asanbyeongwon-Gil, Songpa-Gu, Seoul, 05505, South Korea.
Eur Radiol. 2020 Feb;30(2):735-743. doi: 10.1007/s00330-019-06412-4. Epub 2019 Aug 30.
To determine whether sirolimus has beneficial effects on lymphangioleiomyomatosis (LAM) lung cysts in CT with long-term follow-up (FU) and to investigate whether CT is an appropriate imaging biomarker to monitor and evaluate LAM progression.
In this retrospective study, 73 female patients diagnosed with definite LAM between May 2001 and June 2018 were included. Among these, 39 (53.4%) were treated with sirolimus. Quantitative and qualitative CT scoring for lung cysts (CS) were performed and compared between time points (baseline vs. FU at starting sirolimus, baseline vs. last FU, and FU at starting sirolimus vs. last FU for patients treated with sirolimus; baseline vs. last FU for patients without sirolimus). The correlation between CS at each time point and pulmonary function tests (PFTs) at each time point in the patients treated with sirolimus was also investigated. The quantitative and qualitative analyses and PFT results were compared between time points.
In both quantitative and qualitative analyses, CS significantly increased from baseline to FU after starting sirolimus, and from baseline to last FU (all p < 0.05), whereas there was no significant difference between scores at the start of sirolimus vs. last in the patients treated with sirolimus. After sirolimus treatment, diffusing capacity for carbon monoxide (DL) was significantly increased. There were significant correlations between CS at each time point and PFT (correlation coefficient [r], - 0.383-0.935; all p < 0.001).
Patients with LAM benefited from sirolimus. CT could be a useful imaging biomarker for evaluating and monitoring lung cysts in LAM.
• Qualitative analysis showed a total of 15.8% to 21.1% of patients had a reduced lung cyst volume after sirolimus treatment, and in quantitative analysis, there was no significant difference in lung cyst volume between CT at the start of sirolimus therapy and the last CT. • Pulmonary function was also improved or maintained after sirolimus treatment. • Chest CT could be a useful imaging biomarker for evaluating and monitoring lung cysts in patients with lymphangioleiomyomatosis.
通过长期随访（FU）确定西罗莫司对淋巴管平滑肌瘤病（LAM）肺部囊肿的影响，并探讨 CT 是否为监测和评估 LAM 进展的合适影像学生物标志物。
本回顾性研究纳入 2001 年 5 月至 2018 年 6 月期间诊断为明确 LAM 的 73 名女性患者，其中 39 名（53.4%）接受了西罗莫司治疗。对肺囊肿（CS）的 CT 定量和定性评分进行了评估，并对以下各时间点之间的评分进行了比较（西罗莫司治疗开始时的基线与 FU、基线与最后一次 FU、西罗莫司治疗开始时的 FU 与最后一次 FU；未接受西罗莫司治疗的患者的基线与最后一次 FU）。还研究了接受西罗莫司治疗的患者在每个时间点的 CS 与每个时间点的肺功能测试（PFT）之间的相关性。比较了各时间点的定量和定性分析以及 PFT 结果。
在定量和定性分析中，CS 均从西罗莫司治疗开始时的基线水平增加到 FU，从基线水平增加到最后一次 FU（均 p<0.05），而接受西罗莫司治疗的患者在开始西罗莫司治疗时与最后一次 FU 时的评分之间无显著差异。在接受西罗莫司治疗后，一氧化碳弥散量（DL）显著增加。CS 在每个时间点与 PFT 之间均存在显著相关性（相关系数 [r]，-0.383 至 0.935；均 p<0.001）。
患有 LAM 的患者从西罗莫司治疗中受益。CT 可作为评估和监测 LAM 肺部囊肿的有用影像学生物标志物。
定性分析显示，共有 15.8%至 21.1%的患者在接受西罗莫司治疗后肺囊肿体积减少，在定量分析中，西罗莫司治疗开始时的 CT 与最后一次 CT 的肺囊肿体积无显著差异。
肺功能在接受西罗莫司治疗后也得到改善或维持。
胸部 CT 可作为评估和监测淋巴管平滑肌瘤病患者肺部囊肿的有用影像学生物标志物。
