Outcome of patients admitted to the intensive care unit for acute exacerbation of idiopathic pulmonary fibrosis.

Abstract

The aim of this study was to evaluate the outcome of intensive care unit (ICU) admission in patients with idiopathic pulmonary fibrosis (IPF) who develop acute respiratory failure of unknown etiology. A retrospective study at University of Manitoba hospitals reviewed all patients admitted to the ICU from November 1988 to December 2000 with IPF requiring mechanical ventilation for unknown causes of acute respiratory failure. Survival at hospital discharge was assessed as the primary end point and ICU length of stay as a secondary end point. In the absence of open lung biopsy, major and minor clinical criteria (as per American Thoracic Society statements) were used for the diagnosis of IPF. Infections were ruled out by extensive surveillance cultures and/or bronchoscopy with bronchoalveolar lavage. Eighty-eight charts were reviewed and 25 patients met the inclusion criteria. The mean (+/- SD) age was 69+/-11 years (range 42 to 96 years) and 23 patients were male. With the exception of one survivor who was discharged home, 21 patients died while receiving mechanical ventilation, and three patients died in hospital shortly after ICU discharge (one day, 22 days and 67 days). Intubation and mechanical ventilation were administered to 21 patients, with a mean duration of 11+/-6 days (range two to 27 days); the other four patients were treated with noninvasive ventilation. The average duration of symptoms before ICU admission was 22+/-26 days. All patients were treated with systemic corticosteroids, while eight patients received additional chemotherapy. In the absence of a reversible cause, patients with IPF who develop acute exacerbation of IPF may not benefit from ICU admission and mechanical ventilation. However, it is imperative that a diagnostic workup be performed to rule out an infectious or other reversible cause of respiratory failure before admission to the ICU is denied.