PT - JOURNAL ARTICLE AU - J.W. Song AU - S-B. Hong AU - C-M. Lim AU - Y. Koh AU - D.S. Kim TI - Acute Exacerbation of Idiopathic Pulmonary Fibrosis: Incidence, Risk Factors, and Outcome AID - 10.1183/09031936.00159709 DP - 2010 Jan 01 TA - European Respiratory Journal PG - erj01597-2009 4099 - http://erj.ersjournals.com/content/early/2010/07/01/09031936.00159709.short 4100 - http://erj.ersjournals.com/content/early/2010/07/01/09031936.00159709.full AB - Although acute exacerbation of idiopathic pulmonary fibrosis (IPF) has become well recognized, the reported incidence and outcomes are highly variable, and risk factors are unknown. The aim of this study was to estimate the incidence, risk factors and impact of acute exacerbations and other known causes of rapid deterioration.Retrospective review of 461 patients with IPF (biopsy-proven: 269).The median follow-up period was 22.9 months. Rapid deterioration requiring hospitalization occurred in 163 patients (35.4%), with multiple episodes in 42 patients. Acute exacerbation was the most frequent cause (55.2%), followed by infection. The one-year and three-year incidences of acute exacerbation were 14.2% and 20.7%, respectively. Never having smoked and low forced vital capacity (FVC) were significant risk factors. The in-hospital mortality rate was 50.0%, and the one and five-year survival rates from the initial diagnosis were 56.2% and 18.4%, respectively. Acute exacerbation was a significant predictor of poor survival after the initial diagnosis, along with increased age, low FVC and diffusing capacity (DL,CO), and steroid use with or without cytotoxic therapy.One and three-year incidences of acute exacerbation were 14.2% and 20.7%, respectively. Never having smoked and low FVC were risk factors. Acute exacerbation had a serious impact on the overall survival of the patients with idiopathic pulmonary fibrosis.