Abstract

The prognosis of patients with idiopathic pulmonary fibrosis or fibrosing idiopathic nonspecific interstitial pneumonia undergoing invasive mechanical ventilation (MV) for acute respiratory failure is known to be poor. The issue of life support in these patients needs to be reconsidered in light of changes during the past decade in ventilator settings and in the management of acute exacerbation. We therefore aimed to reassess the prognosis of such patients. We retrospectively assessed the outcomes of all medical patients with idiopathic pulmonary fibrosis or fibrosing idiopathic nonspecific interstitial pneumonia who required invasive MV in 3 university hospitals in the Paris area from January 2002 to April 2009. In total, 27 patients (mean age, 66 ± 12.8 years) required invasive MV in the intensive care unit: 8(30%) were successfully weaned from MV, and 6 and 4 were discharged from the intensive care unit and the hospital, respectively. Survivals for patients who did not undergo lung transplant were 22%, 3.7%, and 3.7%, at 30 days, 6 months, and 12 months, respectively. We confirm that use of invasive MV for acute respiratory failure in patients with idiopathic pulmonary fibrosis or fibrosing idiopathic nonspecific interstitial pneumonia is associated with a high mortality; however, a subset of patients may be discharged alive from the intensive care unit and hospital, providing an opportunity to consider lung transplant in case of eligibility. Our results suggest that invasive MV should not be systematically denied to these patients but discussed on a case-by-case basis.

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