Outcome of Patients with Idiopathic Pulmonary Fibrosis (IPF) Ventilated in Intensive Care Unit. A Retrospective Study from Sweden

Introduction: IPF is a progressive and generally fatal interstitial lung disease of unknown etiology; nearly all patients die within 6 years of diagnosis. Acute respiratory failure (ARF) occurring during. IPF is associated with a poor prognosis Noninvasive ventilation (NIV) can be an effective treatment method to prevent intubation, can reduce mortality in IPF patients.

Material and methods: This is a retrospective analysis of short-term outcomes in IPF patients being administered NIV for ARF. 27 IPF patients who were administered NIV between January 1, 2004, and December 2015, were included. The outcome measures are the need for endotracheal intubation despite NIV treatment and mortality rate during their ICU stay.

Results: Mean and median age was 68.6 and 69.0 years resp. 15 were smokers and 8 former smoker. Mean FEV1 and FEV% was 1,98 and 61.5% respectively. The figures for FVC and FVC% was 2,4 and 58.2% respectively. In all, 14 were admitted to ICU because of respiratory infections, worsening of IPF in 4 (14,8%). Charlson Comorbidity Index was more than 2 in 50% of the patients. NIV was successful in 12 patients and unsuccessful in 15 who required endotracheal intubation. All the patients in the NIV failure group died within 20.2±15.3 days of intubation. Fifteen died at ICU and the others were admitted back to their home clinic. Three patients died during 30 days after charged from ICU.

Conclusions: The outcome of IPF patients who were administered NIV was quite poor. The use of NIV was, nevertheless, found to be associated with clinical benefits in selected IPF patients, preventing the need for intubation and reducing the rate of complications/death.