特发性肺纤维化患者死亡预后因素的回顾性队列研究

目的 探讨特发性肺纤维化(IPF)患者的临床、影像学和肺生理学指标与疾病预后的关系.方法 2000年1月至2004年6月在中国医科大学附属第一医院126例经临床诊断的IPF患者作为研究对象,其中男81例,女45例,年龄51～67岁,平均61岁.采用单因素和多因素Cox比例风险回归分析评估患者临床、呼吸生理、BALF及HRCT等参数的死亡风险度,采用Kaplan-Meier检验比较组间生存率.结果平均随访时间为29.6个月,IPF患者的生存率为46.8%(59/126),确诊后的中位生存期为30个月.采用糖皮质激素和(或)细胞毒类药物治疗与采用其他药物治疗的生存率比较,差异无统计学意义(Ward值为2.3,P>0.05);性别和吸烟状态的组间生存率比较,差异无统计学意义(Ward值为0.11和1.65,均P>0.05).将单因素Cox比例风险回归分析结果中有意义的变量中位数为截点值,进行组间生存率比较,结果表明,呼吸困难评分、FVC、肺总量、DLCO、BALF中性粒细胞、嗜酸粒细胞、胸部HRCT网格影和蜂窝肺的组间生存率差异均有统计学意义(Logrank值为13.52～57.52,均P<0.05).多因素Cox比例风险回归分析结果表明,肺总量、DLCO、HRCT网格影和蜂窝肺均为影响IPF患者预后的因素(Ward值为5.76～74.20,均P<0.01).结论 肺总量、DLCO、BALF细胞学指标和肺纤维化程度是影响IPF患者预后的主要因素,其中肺总量和DLCO与IPF患者的预后呈负相关.糖皮质激素和(或)细胞毒类药物不能改变IPF患者的预后.

A retrospective cohort study of prognostic factors for death in patients with idiopathic pulmonary fibrosis

Objective To investigate the prognostic implications of clinical,radiographic,and physiological variables in idiopathic pulmonary fibrosis (IPF).Methods The clinical,pulmonary physiological,bronchoalveolar lavage fluid (BALF) cell differentials and lung high-resolution computed tomography (HRCT) at diagnosis in 126 patients with IPF were retrospectively analyzed.Univariate and multivariate Cox proportional-hazards regression analysis was used to evaluate various parameters associated with hazard ratio (HR).The survival rates of all groups were compared using the Kaplan-Meier method.Results In 29.6 months of average follow-up time,the survival rate of the IPF patients was 46.8% (59/126),and the median survival time was 30 months after diagnosis.Glucocorticoids and/or cytotoxic drugs for patients with IPF did not change the prognosis.The survival rates between groups by gender and smoking status showed no statistically significant difference (Ward:0.11,1.65,P>0.05).The patients were divided into 2 groups by the median (the cutoff point value)of significant variables in univariate Cox proportional-hazards regression analysis,and the survival rates showed statistically significant difference by dyspnea scale,FVC%,TLC%,DLCO%,neutrophil percentage and eosinophil percentate in BALF,and the reticular and honeycomb lung score (Logrank:13.52-57.52,P<0.05).The results of multivariate Cox proportional-hazards regression analysis showed that TLC%,DLCO%,HRCT reticular score and honeycomb lung score were factors that affected the prognosis of patients with IPF (Wald=5.76-21.48,P<0.05).Conclusions TLC%,DLCO%,cell differentials of BALF and the degree of pulmonary fibrosis were the main factors affecting the prognosis of patients with IPF.TLC% and DLCO% showed a negative correlation with the prognosis of patients with IPF.Glucocorticoids and/or cytotoxic drug therapy had no effect on the prognosis of patients with IPF.