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

• Published in: Chinese medical journal Vol. 132; no. 18; pp. 2177 - 2184
• Main Authors: Cao, Meng-Shu, Sheng, Jian, Wang, Tian-Zhen, Qiu, Xiao-Hua, Wang, Dong-Mei, Wang, Yang, Xiao, Yong-Long, Cai, Hou-Rong
• Format: Journal Article
• Language: English
• Published: China Lippincott Williams & Wilkins Ovid Technologies 20.09.2019; Wolters Kluwer Health; Wolters Kluwer
• Subjects: Acute Disease; Female; Hospitals; Humans; Idiopathic Pulmonary Fibrosis - mortality; Idiopathic Pulmonary Fibrosis - pathology; Male; Medical prognosis; Medical records; Mortality; Multivariate Analysis; Original; Patients; Pneumonia; Prognosis; Proportional Hazards Models; Pulmonary fibrosis; Respiratory Function Tests; Retrospective Studies; United States > US
• ISSN: 0366-6999; 2542-5641; 2542-5641
• DOI: 10.1097/CM9.0000000000000422

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). 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-Whitney U test. Categorical variables were analyzed using χ test. Log-rank test was used for the survival analysis. Cox proportional models evaluated the risk factors for AE occurrence and survival. 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 (χ = 13.567, P < 0.001; z = -2.936, P = 0.003; χ = 5.901, P = 0.015; t = 2.048, P = 0.043; χ = 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 (χ = 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 (χ = 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)/fractional concentration of inspired oxygen (FiO2), 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). 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.