Objective: During the course of idiopathic pulmonary fibrosis patients may need invasive mechanical ventilation because of acute respiratory failure. We reviewed the charts of all patients with idiopathic pulmonary fibrosis admitted to our ICU for mechanical ventilation to describe their ICU course and prognosis.

Design and setting: Retrospective, observational case series, from December 1996 to March 2001, in an 18-bed medical ICU in a tertiary university hospital.

Patients: Fourteen consecutive patients with idiopathic ( n=11) or secondary ( n=3) pulmonary fibrosis admitted to the medical ICU for mechanical ventilation.

Measurements and results: Relevant factors of history and hospital course such as diagnostic and therapeutic interventions were retrieved as well as laboratory and radiological results. All patients were admitted for severe acute hypoxemic respiratory failure (PaO(2)/FIO(2) 111+/-64 mmHg), with a high clinical suspicion of lower respiratory tract infection. Despite ventilatory support and adjunctive therapies (antibiotics, steroids, or immunosuppressive drugs), all patients gradually worsened and eventually died in the ICU after a mean stay of 7.6+/-4.6 days.

Conclusions: In this study mechanical ventilation for acute respiratory failure in pulmonary fibrosis patients was associated with a 100% mortality, despite aggressive therapeutic and diagnostic procedures.