Background: Acute exacerbation (AE) is the major cause of morbidity and mortality in patients with idiopathic\npulmonary fibrosis (IPF). AEs also occur in other forms of fibrosing interstitial lung disease (fILD). The clinical features\nand prognosis of AE patients with connective tissue diseases (CTDs) associated-ILD has not been fully described.\nMethods: We retrospectively reviewed 177 patients with either IPF or a characterized CTD-ILD admitted to Nanjing\nDrum Tower Hospital with an AE from January 2010 to December 2016.\nResults: The study cohort included 107 subjects with AE-IPF and 70 cases with AE-CTD-ILD. Female gender, prior\nuse of corticosteroid and immunosupressants, lower serum albumin, higher D-dimer level, TLC% pred, survival, and\ntreatment with immunosupressants and caspofungin were more common in the CTD-ILD group (all p<0.05). The\nincidences of AE-CTD-ILD and AE-IPF were similar in our single center (p = 0.526). TLC% pred was the risk factor for\nAE after ILD diagnosis for 1 year in CTD patients (p = 0.018). Log-rank tests showed patients with CTD-ILD had a\nsignificantly lower mortality rate compared with IPF patients after AEs (p = 0.029). No significant difference in\nsurvival was noted among CTD subgroups (p = 0.353). The survival was negatively correlated with WBC count, LDH\nand CT score, (p = 0.006, p = 0.013 and p = 0.035, respectively), and positively correlated with PaO2/FiO2 ratio (p<\n0.001) in the CTD-ILD group. WBC count and PO2/FiO2 ratio were the independent predictors for survival in AECTD-\nILD after adjusting for other clinical variates in Cox regression Models (p = 0.038 and p < 0.001, respectively).\nConclusions: The clinical characteristics of patients with AE-CTD-ILD differed from those with AE-IPF, while AE\nincidences were similar between the two groups. Subjects with AE-CTD-fILD tended to have a better prognosis,\nand WBC count and PO2/FiO2 ratio were the independent survival predictors for these patients.
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