目的 探讨特发性肺纤维化(IPF)患者和结缔组织病相关性纤维化间质性肺疾病(CTD-fILD)患者急性加重(AE)的短期内死亡的危险因素。方法 回顾性分析2017年10月—2019年9月在深圳大学和广州医科大学附属第一医院住院的25例 AE-CTD-fILD和26例AE-IPF患者临床信息,Kaplan-Merier法对两组患者进行生存分析,Cox回顾分析年龄、性别、吸烟、白细胞总数、C反应蛋白、红细胞沉降率及肿瘤指标在急性加重患者死亡中的作用。结果 与AE-CTD-fILD比较,AE-IPF患者组男性比例、年龄、吸烟比例较高,红细胞沉降率较低(24/26 vs 10/25,P<0.001;63.77±9.97 vs 58.00±10.32,P=0.048;16/26 vs 9/25,P=0.02;28.07±29.45 vs 64.35±40.34,P=0.002 );90天内,26例AE-IPF患者11例死亡,25例AE-CTD-fILD患者5例死亡,死亡率无明显差异(42.3% vs 20%,P=0.073);Cox回归分析显示,白细胞计数是AE-IPF和AE-CTD-fILD患者的死亡危险因素(HR=1.305,P=0.001;HR=1.529,P=0.009);CA15-3是AE-IPF患者死亡危险因素(HR=1.015,P=0.005)。结论 急性加重IPF和CTD-fILD患者短期内死亡风险相似,白细胞计数及外周CA15-3水平可能是肺纤维化急性加重患者短期内死亡的危险因素。

Objective To explore the risk factors for acute exacerbation (AE) in patients with idiopathic pulmonary fibrosis (IPF) and connective tissue disease associated with fibrotic interstitial lung disease (CTD-fILD). Methods We retrospectively reviewed 25 patients with AE-CTD-fILD and 26 patients with AE-IPF, and Kaplan-Merier was used to analyze the survival of the two groups of patients. The impact of age,gender, smoking,WBC,CRP,ESR and tumor markers on acute exacerbation death were performed by Cox regression analysis. Results The AE-IPF patients had a higher proportion of men,age and smoking,and a lower ESR compared with AE-CTD-fILD patients(24/26 vs 10/25,P<0.001;63.77±9.97 vs 58.00±10.32,P=0.048;16/26 vs 9/25,P=0.02;28.07±29.45 vs 64.35±40.34,P=0.002 ). 11 cases of 26 patients with AE-IPF and 5 cases of 25 patients with AE-CTD-fILD died within 90 days, Log-rank tests showed patients with CTD-fILD had similar mortality rate compared with IPF patients after AE(42.3% vs 20%,P=0.073). The WBC count was negatively correlated with survival and the independent predictors for patients with AE-IPF and AE-CTD-fILD after adjusting for other clinical variates in Cox regression models(HR=1.305,P=0.001;HR=1.529,P=0.009). CA15-3 may be a risk factor for death of AE-IPF patients(HR=1.015,P=0.005). Conclusion AE-CTD-fILD and AE-IPF were associated with similar poor short-term survival, WBC count and plasma CA15-3 may be the independent survival predictors respectively for patients with acute exacerbation of pulmonary fibrosis in short term.