Noninvasive ventilation in acute exacerbation of idiopathic pulmonary fibrosis.

BACKGROUND AND OBJECTIVE: The outcome of acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is usually very poor, and it has been suggested that mechanical ventilation does not benefit AE-IPF patients. Noninvasive ventilation (NIV) has attracted attention as a means to avoid intubation in acute respiratory failure, including acute respiratory distress syndrome (ARDS). This study describes the outcome of patients with AE-IPF who were treated with NIV.

METHODS: Patients included in the study were those who fulfilled the criteria for AE-IPF during the periods between April 1998 and June 2004 at Tosei General Hospital, and in whom NIV was introduced. Clinical data were obtained retrospectively from patient records.

RESULTS: This study included 11 patients. The initial NIV settings were continuous positive airway pressure (CPAP) mode in 6 patients (mean 10.1+/-2.5 cmH(2)O) and Spontaneous/Timed mode in 5 (mean inspiratory positive airway pressure/expiratory positive airway pressure; 15.0+/-3.3/10.2+/-2.9 cmH(2)O). Five patients avoided intubation and survived more than 3 months after AE-IPF. Six patients who failed NIV died within 3 months. In these 6 patients, 4 required intubation. The other 2 patients, who refused endotracheal intubation, died without intubation. Median survival time and 3-month survivals after acute exacerbation were 30 days, and 45.5%, respectively.

CONCLUSION: Considering extremely poor prognosis of AE-IPF, our findings suggest that NIV is a viable option for the respiratory management in AE-IPF, and should be studied in a large, well-controlled trial.