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Acute exacerbation of idiopathic pulmonary fibrosis: usual interstitial pneumonitis vs.possible usual interstitial pneumonitis pattern

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Author:: No author available

Journal Title:: Chinese Medical Journal

Issue:: 18

DOI:: 10.1097/CM9.0000000000000422

Key Word:: Idiopathic pulmonary fibrosis;Acute exacerbation;Usual interstitial pneumonitis

Abstract： 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.

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