Acute Exacerbation of Idiopathic Pulmonary Fibrosis by New Definition : Incidence, Risk Factors and Outcome = Acute Exacerbation of Idiopathic Pulmonary Fibrosis by New Definition : Incidence, Risk Factors and Outcome

다국어 초록 (Multilingual Abstract)

Background: In 2016, a new conceptual framework for acute respiratory deterioration (ARD) and a revised definition of acute exacerbation (AE) in idiopathic pulmonary fibrosis (IPF) were proposed. The aim of this study was to investigate the incidence, risk factors and outcome of AE in IPF by a revised definition and compare with the results of past definition.
Methods: Patients with IPF (n=445) were included and clinical data were retrospectively reviewed.
Results: The median follow-up period was 30.6 months. ARD requiring hospitalization occurred in 28.9% and AE in 17.3 %. The 1- and 3-year incidences of AE were 7.6 and 20.9%, respectively. AE group was older and had lower FVC, DLco and the lowest, resting oxygen saturation and distance during of 6-minute walk test (6MWT) and more decline in FVC (for 6 months), compared to non-ARD group. Age, DLco and decline in FVC were significant predictors for AE. The in-hospital mortality rate after AE was 29.4%, and PaO2/FiO2 ratio and procalcitonin levels were significant prognostic factors. Compared to results of the past definition (1- year incidence, 5.2%; in-hospital mortality rate, 41.3%), the revised definition increased the incidence by 46%, and decreased in-hospital mortality rate by 28.8%.
Conclusion: The revised definition significantly changed the incidence and outcome of AE. Old age, low DLco and more decline in FVC were risk factors for AE.