Noninvasive ventilation in acute exacerbation of idiopathic pulmonary fibrosis

Intern Med. 2010;49(15):1509-14. doi: 10.2169/internalmedicine.49.3222. Epub 2010 Aug 2.


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.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acute Disease
  • Aged
  • Aged, 80 and over
  • Female
  • Humans
  • Idiopathic Pulmonary Fibrosis / mortality
  • Idiopathic Pulmonary Fibrosis / physiopathology
  • Idiopathic Pulmonary Fibrosis / therapy*
  • Male
  • Middle Aged
  • Respiration, Artificial / methods*
  • Survival Rate / trends
  • Treatment Outcome