Lymphangioleiomyomatosis Screening in Women With Tuberous Sclerosis
Lymphangioleiomyomatosis Screening in Women With Tuberous Sclerosis
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DOI：
10.1378/chest.12-2813
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发表时间：
2013-08-01
期刊：
影响因子：
9.6
通讯作者：
Young, Lisa R.
中科院分区：
文献类型：
--
作者：
Cudzilo, Corey J.;Szczesniak, Rhonda D.;Young, Lisa R.
背景：根据胸部 CT 扫描结果诊断，至少 40% 患有结节性硬化症 (TSC) 的女性患有淋巴管平滑肌瘤病 (LAM)。早期识别可以为生活方式选择和治疗决策提供信息。在这里，我们报告了一家大型 TSC 诊所的 LAM 患病率，并提出了一种对 TSC 女性患者进行 LAM 的 CT 扫描筛查方法。方法：我们回顾性回顾了 12 年期间在我们中心就诊的所有 15 岁 TSC 女性患者的初始胸部 CT 扫描。对每个 CT 图像切片手动评分是否存在特征性薄壁囊肿，如果所有光泽上的囊肿总和超过 3 个囊肿，则诊断为 LAM。 结果：在 133 名女性 TSC 患者中，101 名进行了胸部 CT 扫描以供复查。 48 名 (47.5%) 在初次 CT 扫描中符合 TSC-LAM 标准。 LAM 的风险与年龄有关，每年增加约 8%。 40 岁受试者中 LAM 的患病率为 27%。在患有 LAM 的无症状受试者中，84% 的患者在隆突水平的单张图像中存在囊肿。大多数患有 LAM 的受试者最终出现肺部症状 (63%)，12.5% 死于 LAM。结论：这些结果表明，大多数患有 TSC 的女性最终会出现与 LAM 一致的囊性变化，并且大多数病例可以通过隆突水平的单个 CT 成像切片来识别。 TSC-LAM 与该转诊人群中明显的发病率和死亡率相关。使用有限 CT 扫描方法的基于年龄的方法可能有助于筛查和随后的治疗决策，同时减少该高危人群的辐射暴露。
Background: Lymphangioleiomyomatosis (LAM) occurs in at least 40% of women with tuberous sclerosis complex (TSC), as diagnosed based on chest CT scan findings. Early identification may inform lifestyle choices and treatment decisions. Here we report LAM prevalence in a large TSC clinic and propose an approach to CT scan screening for LAM in women with TSC.Methods: We retrospectively reviewed initial chest CT scans of all female patients with TSC aged 15 years seen at our center over a 12-year period. Each CT image slice was manually scored for the presence or absence of characteristic thin-walled cysts, and the diagnosis of LAM was made if the sum of the cysts on all sheen exceeded three cysts.Results: Of 133 female patients with TSC, 101 had chest CT scans available for review. Forty-eight (47.5%) met criteria for TSC-LAM on the initial CT scan. The risk of LAM was age dependent, rising by about 8% per year. The prevalence of LAM was 27% in subjects 40 years of age. Among asymptomatic subjects with LAM, 84% had cysts present in the single image at the level of the carina. Most subjects with LAM eventually developed pulmonary symptoms (63%), and 12.5% died from LAM.Conclusions: These results suggest that most women with TSC ultimately develop cystic changes consistent with LAM and that most cases can be identified from a single CT imaging slice at the level of the carina. TSC-LAM was associated with appreciable morbidity and mortality in this referral population. An age-based approach using limited CT scanning methods may facilitate screening and subsequent treatment decisions with decreased radiation exposure in this at-risk population.