Kaplan-Meier survival analysis showing significantly higher mortality of AE-IPF than IPF (stable IPF) by using the log-rank test (p = 0.039). AE-IPF patients (39.5%) had higher one-year mortality than stable IPF (28.7%) (p = 0.041). In AE-IPF group (23 cases of death), 19 cases died of respiratory failure by acute exacerbation; 4 died of severe pneumonia. In stable IPF group (50 cases of death), 15 died of acute exacerbation in the period of follow-up, 10 died of pneumonia, 5 died of lung cancer, 2 died of liver cancer, 2 died of intestine cancer, 5 died of myocardial infarction, 2 died of gastrointestinal bleeding, 4 died of pulmonary embolism, 3 died of stroke, 1 died of diabetes, and 1 died of trauma.