Objective: To evaluate outcomes of patients admitted to an intensive care unit with idiopathic pulmonary fibrosis (IPF) and acute respiratory deterioration.
Design: Retrospective cohort study.
Setting: University-affiliated, tertiary adult ICU.
Participants: All patients admitted to the ICU between January 1996 and December 2006 with an established diagnosis of IPF were identified from review of case notes, ICU charts and electronic hospital records. Patient demographics, ICU investigation and treatment profiles were characterised; final follow-up was electronically from the State Registry.
Main outcome measures: Primary study end-point was survival from ICU admission until December 2007; secondary end-points were survival from diagnosis and from first respiratory hospital admission.
Results: 24 patients were identified with IPF, diagnosed by lung biopsy (8) and by clinical criteria; 21 patients were admitted with acute respiratory failure, and three postoperatively. Mean age was 66 (SD, 16) years, mean APACHE III score was 82 (SD, 37), and 14 were men. Admission diagnoses were acute exacerbation of IPF (8), pneumonia (10), acute heart failure (3) and postoperative (3). All patients received broad-spectrum antibiotics, and 17 continued to receive corticosteroids prescribed previously. Nineteen patients were mechanically ventilated for a mean of 19.1 days (SD, 25.3); 13 of 19 patients had non-invasive ventilation before mechanical ventilation, and four received only non-invasive ventilation. Sixteen mechanically ventilated patients died in the ICU, another six died in hospital (2-30 days after ICU discharge); and two were discharged home. Median survival (95% CI) was 16 (9-19) days from ICU admission; 55 (18-284) days from first respiratory admission; and 1.05 (0.34-1.75) years from IPF diagnosis.
Conclusions: Outcomes of patients with IPF admitted to the ICU are poor. Indications for mechanical ventilation appear uncertain.