Decline in lung function in patients with lymphangioleiomyomatosis treated with or without progesterone
Decline in lung function in patients with lymphangioleiomyomatosis treated with or without progesterone
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DOI：
10.1378/chest.126.6.1867
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发表时间：
2004-12-01
期刊：
影响因子：
9.6
通讯作者：
Moss, J
中科院分区：
文献类型：
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作者：
Taveira-DaSilva, AM;Stylianou, MP;Moss, J
研究目的：淋巴管肌瘤病(LAM)是一种影响女性并引起肺囊性病变的疾病，在某些情况下会导致呼吸衰竭和死亡，雌激素似乎会加重这种疾病。因此，黄体酮的激素治疗经常被使用：然而，疗效尚未被证明。我们的目的是确定黄体酮治疗是否延缓了LAM患者肺功能的下降。设计：回溯性研究。设计：回溯性研究。单位：马里兰州贝塞斯达国立卫生研究院。设计和对象：研究人群包括348名I-A-M患者，他们参加了一项纵向研究方案。对275例患者进行了大约4年的观察，测量了肺对一氧化碳(DLCO)和FEV1的扩散能力的下降。用黄体酮、po(n=67)或im(n=72)治疗的患者与未治疗的患者(n=136)比较DLCO和FEV1的下降。测量和结果：DLCO和FEV1的年下降速度分别为2.4+/-0.4%预测(0.69+/-0.07mL/min/mm Hg)和1.7+/-0.4%预测(75+/-9mL)(平均扫描电子显微镜)。功能下降的最显著预测因素是初始肺功能和年龄。在对初始FEV1、年龄和病程进行调整后。接受孕酮治疗的患者的FEV1下降率往往低于口服治疗的患者(分别为1.9%+/-0.6%和3.2%/-0.8%，p=0.081)。然而。肌注黄体酮的患者和未治疗的患者的FEV_1下降率无显著差异(1.9+/-0.6%对0.8+/-0.5%的预测；p=0.520)；口服黄体酮的患者和未治疗的患者的FEV_1下降率(3.2+/-0.8%，预测对0.8+/-0.5%的预测；p=0.064)。在对初始DLCO进行调整后。口服黄体酮治疗的患者DLCO下降率显著更高(预计为3.6+/-0.7%)。P=0.002)和IM孕酮(2.8%+/-0.5%预测p=0.022)高于未治疗患者(1.6%/-0.6%预测)。结论：在回顾性研究的限制范围内，我们的数据提示孕酮治疗不能减缓LAM患者肺功能的下降。
Study objective: Lymphangioleiomyomatosis (LAM), a disease affecting women and causing cystic lung lesions, and, in some instances, leading to respiratory failure and death, appears to be exacerbated by estrogens. Hence, hormonal therapy with progesterone is frequently employed: however, efficacy has not been demonstrated. Our aim was to determine whether progesterone administration slowed the decline in lung function in LAM. Design: Retrospective study.Design: Retrospective study.Setting: National Institutes of Health, Bethesda, MD. Design and subjects: The study population comprised 348 patients with I-A-M participating in a longitudinal research protocol. Declines in diffusion capacity of the lung for carbon monoxide (DLCO) and FEV1 were measured in 275 patients observed for approximately 4 years. The declines in DLCO and FEV1 of patients treated with progesterone, po (n = 67) or IM (n =72) were compared with those of untreated patients (n = 136).Measurements and results: Overall yearly rates of decline in DLCO and FEV1 were 2.4 +/- 0.4% predicted (0.69 +/- 0.07 mL/min/mm Hg) and 1.7 +/- 0.4% predicted (75 +/- 9 mL), respectively (mean SEM). The most significant predictors of functional decline were initial lung function and age. After adjusting for initial FEV1, age, and duration of disease.. patients treated with LM progesterone tended to have lower rates of decline in FEV1 than patients treated po (1.9 +/- 0.6%, predicted vs 3.2 +/- 0.8% predicted, respectively, p = 0.081). However. there was no significant difference in rates of decline in FEV1 between patients treated with IM progesterone and untreated patients (1.9 +/- 0.6% predicted vs 0.8 +/- 0.5% predicted, respectively; p = 0.520): and patients treated with po progesterone and untreated patients (3.2 +/- 0.8%, predicted vs 0.8 +/- 0.5% predicted, respectively; p = 0.064). After a adjusting for initial DLCO. rates of decline in DLCO were significantly higher in patients treated with po progesterone (3.6 +/- 0.7% predicted. p = 0.002) and IM progesterone (2.8 +/- 0.5% predicted p = 0.022) than in untreated patients (1.6 +/- 0.6% predicted).Conclusions: Within the limitations of a retrospective study, our data suggest that progesterone therapy does not slow the decline in lung function in LAM.