西罗莫司降低淋巴管肌瘤病患者气胸复发的风险：一项历史性前瞻性自身对照研究。
Sirolimus reduces the risk of pneumothorax recurrence in patients with lymphangioleiomyomatosis: a historical prospective self-controlled study.
机构信息
State Key Laboratory of Complex Severe and Rare Diseases, Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
出版信息
Orphanet J Rare Dis. 2022 Jul 8;17(1):257. doi: 10.1186/s13023-022-02418-2.
BACKGROUND
Spontaneous pneumothorax has a high incidence and high rate of recurrence in patients with lymphangioleiomyomatosis (LAM). The risk factors for pneumothorax and the effects of sirolimus on pneumothorax in patients with LAM are unknown. In our study, multivariate logistic regression was applied to a cross-sectional cohort to investigate factors associated with pneumothorax in LAM patients. Kaplan-Meier analysis was applied in the historical prospective self-controlled study to determine whether sirolimus reduces the risk of pneumothorax recurrence in patients with LAM.
RESULTS
Of the 399 patients registered with LAM-CHINA at our center between May 10, 2017 and August 31, 2020, 142 had a history of pneumothorax at registration. High CT grade and age at presentation ≤ 35 years were associated with a higher risk of pneumothorax in patients with LAM. Postmenopausal status was correlated with a lower risk of pneumothorax. In the historical prospective self-controlled study, the 5-year probability of pneumothorax recurrence was 80% lower in the sirolimus group than in the control group (hazard ratio for pneumothorax recurrence, 0.20; 95% CI, 0.14 to 0.30, P < 0.001 by log-rank test).
CONCLUSION
Sirolimus reduced the risk of pneumothorax recurrence in LAM patients.
背景
淋巴管肌瘤病（LAM）患者的自发性气胸发病率和复发率均较高。气胸的危险因素以及西罗莫司对 LAM 患者气胸的影响尚不清楚。在我们的研究中，采用多变量逻辑回归对横断面队列进行分析，以探讨与 LAM 患者气胸相关的因素。采用 Kaplan-Meier 分析对历史前瞻性自身对照研究进行分析，以确定西罗莫司是否降低 LAM 患者气胸复发的风险。
结果
在 2017 年 5 月 10 日至 2020 年 8 月 31 日期间在我们中心登记的 399 例 LAM-CHINA 患者中，有 142 例在登记时患有气胸。高 CT 分级和发病时年龄≤35 岁与 LAM 患者气胸风险较高相关。绝经后状态与气胸风险降低相关。在历史前瞻性自身对照研究中，与对照组相比，西罗莫司组气胸复发的 5 年概率降低了 80%（气胸复发的风险比，0.20；95%CI，0.14 至 0.30，log-rank 检验 P<0.001）。
结论
西罗莫司降低了 LAM 患者气胸复发的风险。
Zotero 插件