The characteristics and outcomes of patients with idiopathic pulmonary fibrosis admitted to the ICU with acute respiratory failure

•Hospital mortality rate for IPF patients with acute respiratory failure is 43.8%•Hospital mortality rate for IPF patients requiring ventilator support is 56.7%•Outcomes are poor in those with IPF exacerbations requiring ventilator support To date, studies have provided conflicting results regarding the outcomes of patients with Idiopathic Pulmonary Fibrosis (IPF) admitted to the ICU with acute respiratory failure (ARF). To understand the characteristics and outcomes of these patients. Retrospective study using a large single-center ICU database. We identified 48 unique patients with IPF admitted for ARF from 2001-2012. The most common causes of ARF were IPF exacerbation and pneumonia. The overall hospital mortality rate was 43.8% and was 56.7% in those who required invasive mechanical ventilation (IMV). In patients requiring IMV for IPF exacerbation, the mortality rate was 81.3%. In multiple regression analysis, the presence of diabetes mellitus was associated with decreased mortality whereas the need for IMV was associated with increased mortality. Although the overall mortality rate for IPF patients with ARF has improved, the need for IMV due to IPF exacerbations is associated with increased mortality.