摘要
Background:The prognosis of acute exacerbation of idiopathic pulmonary fibrosis(AE-IPF)is very poor with a high mortality.The aim of this study was to describe the clinical features and survival of patients with AE-IPF with usual pulmonary fibrosis(UIP)and possible UIP(P-UIP)pattern on chest high resolution computed tomography(HRCT).Methods:This retrospective study included 107 patients with AE-IPF admitted to Nanjing Drum Tower Hospital from January 2010 to December 2016.The subjects were divided into UIP(n=86)and P-UIP group(n=21)based on chest HRCT.Continuous variables were analyzed using Student's t test or Mann-Whimey U test.Categorical variables were analyzed using x2 test.Log-rank test was used for the survival analysis.Cox proportional models evaluated the risk factors for AE occurrence and survival.Results:The male,older patients,previous N-acetylcysteine use,elevated white blood cell(WBC)counts,and microbiology infection were more common in the UIP group than the P-UIP group(X2=13.567,P<0.001;z=-2.936,P=0.003;X2=5.901,P=0.015;t=2.048,P=0.043;x2=10.297,P=0.036,respectively).The percentage of AE with UIP pattern in idiopathic interstitial pneumonia(ⅡP)was significantly higher than P-UIP pattern(X2=40.011,P<0.001).Smoking was the risk factor for AE within 6 months after IPF diagnosis in the UIP group.The cumulative proportion survival of 30-days was significantly higher in the UIP group compared with the P-UIP group(x2=5.489,P=0.019)despite of the similar overall survival in the two groups.Multivariate Cox regression analysis indicated WBC count,partial pressure of oxygen in artery(PaO2)/ffactional concentration of inspired oxygen(FiOz),and computed tomography(CT)score were the independent predictors for survival in the UIP group(hazard ratio[HR]:1.070,95%confidential interval[CI]:1.027-1.114,P=0.001;HR:0.992,95%CI:0.986-0.997,P=0.002;and HR:1.649,95%CI:1.253-2.171,P<0.001,respectively).Conclusions:AE occurrence of UIP patients in IIP was significantly more than P-UIP cases.The short-term survival was better in the UIP group despite of the similar overall survival in the two groups.WBC count,PaO2/FiO2,and CT score were the independent predictors for survival in UIP subjects.
基金
partially supported by the grants from the National Natural Science Foundation of China(No.81200049 and No.81670059)
the Nanjing Medical Science and Technique Development Foundation(No.ORX17005).
