环磷酰胺治疗特发性肺纤维化的疗效观察

Study on the effects of cyclophosphamide therapy in idiopathic pulmonary fibrosis

目的:观察静脉注射环磷酰胺对特发性肺纤维化的治疗效果。方法:46名患者分为2组,对照组采用为期24个月的标准方案:泼尼松联合口服硫唑嘌呤治疗。观察组采用试验性治疗方案:口服泼尼松和静脉注射环磷酰胺(每月6次和每3个月6次)进行治疗。主要观察指标是36个月内的存活或不需要进行肺移植。次要指标是患者的最大肺活量,对一氧化碳弥散能力,以及在3、6、12、18、24、30、36个月中的基线动脉氧分压。结果:对照组有25名患者,观察组有21名患者,36个月内存活同时未进行移植的患者在对照组中为44%,在观察组中为76%(P=0.028)。而最大肺活量观察组(6.7%)与对照组(8.8%)无统计学差异(P=0.16)。对照组患者中一氧化碳弥散能力下降11.81%,而观察组患者下降4.4%(P=0.056 9)。动脉氧分压无显著性差异。结论:静脉注射环磷酰胺治疗显著提高了肺纤维化患者的存活率。但在肺功能参数和气体交换方面与标准方案没有显著差异。

OBJECrlVE To observe the therapeutic effects of intravenous injection of cyclophosphamide in idiopathic pulmo nary fibrosis. METHODS 46 patients were divided into two groups, the patients of control group were received a period of 24 months of standard scheme： prednisone combined with oral azathioprine. In observer group, the patients were administered with oral prednisone and intravenous injection of cyclophosphamide （6 times per one month and 6 times every three months）. The primary indicator was survival or unneeded lung transplant in 36 months. Vital capacity of the patients, diffusing capacity for carbon monoxide, and partial pressure of arterial oxygen were defined as the secondary indicator at baseline, 3, 6, 12, 18, 24, 30 and 36 months. RESULTS Survival or un-transplant patients in control group （n = 25） and observer group （n = 21） was 44 % and 76%, respectively （P = 0. 028） The vital capacity was worse in observer group （6.7 % ）, comparing to control group （8. 8%） （P = 0. 16）. Carbon monoxide diffusing capacity was decreased 11.81 % in observer group patients and decreased 4. 4% in control group （P = 0. 056 9）. There was no significant difference in arterial partial pressure of oxygen. CONCLUSION Intravenousinjection of cyclophosphamide significantly improved survival in patients with pulmonary fibrosis. But the lung function parameters and gas exchange did not present significant difference as compared to standard scheme.