Background and objective: Previous reports on the outcome of patients with pulmonary fibrosis admitted to the intensive care unit (ICU) suggest a bad prognosis. The aim of our study was to evaluate the course and prognosis of patients with pulmonary fibrosis admitted to the ICU of our hospital because of acute respiratory failure.
Patients and method: Retrospective, case-series, observational study. We evaluated the clinical records of patients with pulmonary fibrosis referred to the intensive care unit in a tertiary university teaching hospital between January 1986 and June 2002. Complete information on the diagnosis and clinical course of the pulmonary disease, pulmonary function tests, current clinical status, ventilatory support and adjunctive therapies applied in the ICU, length of stay and mortality was collected.
Results: Twenty patients were included, 14 with idiopathic pulmonary fibrosis and 6 with pulmonary fibrosis and associated collagen vascular diseases. The mean (SD) interval between the diagnosis of fibrosis and the admission to ICU was 14 (20) months. All patients presented with severe acute respiratory failure (PaO2/FiO2 < 200). The cause of clinical worsening was identified in 8 (40%) cases (5 bacterial and 3 fungal infections). In the ICU, 17 patients required mecanical ventilation. All patients worsened progressively, with refractory hypoxemic respiratory failure (100%) and hemodynamic instability after endotraqueal intubation (70%). Mechanical ventilation was associated with a 100% mortality.
Conclusion: An identifiable cause of acute respiratory failure could not be found in a significant proportion of patients despite a systematic work-up. Mechanical ventilation and aggressive life support measures do not seem to provide any further benefit in patients with pulmonary fibrosis and severe respiratory failure.