Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis
Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis
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DOI：
10.7326/0003-4819-132-12-200006200-00004
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发表时间：
2000-06-20
影响因子：
39.2
通讯作者：
Wise, RA
中科院分区：
文献类型：
--
作者：
White, B;Moore, WC;Wise, RA
背景：肺部炎症（肺泡炎）可能导致硬皮病肺纤维化。目的：确定环磷酰胺治疗是否与硬皮病肺泡炎患者的肺功能保留和生存改善相关。设计：回顾性队列研究，地点：约翰霍普金斯大学和马里兰大学硬皮病中心。患者：103 名接受支气管肺泡灌洗或肺活检的硬皮病患者。干预：环磷酰胺治疗。测量：1) 连续测量用力肺活量 (FVC) 和一氧化碳弥散能力，2) 生存率。结果：在支气管肺泡破坏或活检后中位随访 13 个月期间，未接受环磷酰胺治疗的肺泡炎患者的 FVC 下降（平均差，-0.28 L [95% Cl，-0.41 至 -0.16 L]和预测值的-7.1% [CI，-10.9% 至-4.0%]）。这些患者的一氧化碳弥散能力也下降（平均差为 -3.3 mmol min(-1).kPa(-1) [Cl，-4.6 至 -2.1 mmol.min(-1).kPa(-1)] 和预测值的 -9.6% [CI，-16.7% 至 -2.4%]）。在中位随访 16 个月期间，接受环磷酰胺治疗的肺泡炎患者更有可能在 FVC（相对风险，2.5 [CI，1.5 至 4.1]）和弥散性反应（相对风险，1.5 [CI，1.0 至 2.2]）方面获得良好结果（稳定或改善）。这些患者的生存率也有所提高；未治疗患者的中位生存率为 89%（第 25、75 个百分位数、84%、94%），而未经治疗的患者的中位生存率为 71%（第 25、75 个百分位数、55%、86%）（P = 0.01，对数秩检验）。结论：肺部炎症的存在表明硬皮病患者的肺功能更可能恶化。接受环磷酰胺治疗的肺泡炎患者的肺功能结果和生存率得到改善。
Background: Lung inflammation (alveolitis) may cause lung fibrosis in scleroderma.Objective: To determine whether cyclophosphamide treatment is associated with retention of lung function and improved survival in scleroderma patients with alveolitis.Design: Retrospective cohort study, Setting: Johns Hopkins and University of Maryland Scleroderma Center.Patients: 103 patients with scleroderma who had bronchoalveolar lavage or lung biopsy.Intervention: Cyclophosphamide therapy.Measurements: 1) Serial measurement of forced vital capacity (FVC)and carbon monoxide diffusing capacity and 2) survival.Results: During a median follow-up of 13 months after bronchoalveolar ravage or biopsy, patients with alveolitis who did not receive cyclophosphamide therapy experienced a decrease in FVC (mean difference, -0.28 L [95% Cl, -0.41 to -0.16 L] and -7.1% of the predicted value [CI, -10.9% to -4.0%]). Carbon monoxide diffusing capacity also decreased in these patients (mean difference, -3.3 mmol min(-1).kPa(-1) [Cl, -4.6 to -2.1 mmol.min(-1).kPa(-1)] and -9.6% of the predicted value [CI, -16.7% to -2.4%]). During a median follow-up of 16 months, patients with alveolitis who received cyclophosphamide we re more likely to have a good outcome (stabilization or improvement) in FVC (relative risk, 2.5 [CI, 1.5 to 4.1]) and diffusing rapacity (relative risk, 1.5 [CI, 1.0 to 2.2]). These patients also had improved survival; the median survival rate was 89% (25th, 75th percentiles, 84%, 94%) compared with 71% (25th, 75th percentiles, 55%, 86%) in untreated patients (P = 0.01, log-rank test).Conclusions: The presence of lung inflammation identifies patients with scleroderma who are more likely to have worsening lung function. Lung function outcomes and survival are improved in patients with alveolitis who receive cyclophosphamide.