TY - JOUR T1 - A large cohort study of clinical outcomes and prognostic predictors in patients with acute exacerbation of idiopathic pulmonary fibrosis JF - European Respiratory Journal JO - Eur Respir J VL - 44 IS - Suppl 58 SP - 4627 AU - Seishu Hashimoto AU - Yoshio Taguchi AU - Takashi Hajiro AU - Ikkoh Yasuda AU - Yoshiki Ishii AU - Masahito Ebina AU - Kazuma Kishi AU - Hiroyuki Taniguchi AU - Takafumi Suda AU - Sakae Homma AU - Yukihiko Sugiyama Y1 - 2014/09/01 UR - http://erj.ersjournals.com/content/44/Suppl_58/4627.abstract N2 - Background: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) has been well documented. However, there are a few reports about its clinical outcomes and prognostic predictors.Objectives: To assess clinical outcomes and prognostic predictors in patients with AE-IPF in a multi-institutional cohort.Methods: We retrospectively analyzed 215 consecutive patients who had a diagnosis of AE-IPF between January 2011 and December 2012 at 68 hospitals in Japan. AE-IPF was defined using the revised Japanese criteria. The clinical features, and clinical outcomes and prognostic predictors of AE-IPF were investigated.Results: The overall, 30-day and 90-day mortality rate were 67.0%, 27.4% and 44.7%, respectively. The median survival of patients with AE-IPF was 102 days (95% CI, 74 to 154 days). C reactive protein, lactate dehydrogenase (LDH) and D-dimer in non-survivors at the time of AE were significantly higher than in survivors. In non-survivors at stable status of IPF just before AE, the modified Medical Research Council (mMRC) dyspnea scale was higher, and forced vital capacity (FVC) was lower than in survivors. The initial management was effective in survivors compared with non-survivors. The multivariate Cox analysis revealed that high LDH at the time of AE, and high mMRC dyspnea scale and low FVC at stable status of IPF just before AE were three meaningful predictors of poor survival. The effectiveness of the initial management of AE was related survival.Conclusions: The outcome of AE-IPF was still poor, but better than predicted. Understanding possible prognostic factors could contribute to the better management of AE-IPF. ER -