Respiratory Failure in a Patient with Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis, in its advanced stages, commonly causes chronic respiratory failure. Additionally, patients with fibrotic lung disease can develop acute respiratory failure due to a myriad of causes, though one of the most common and most deadly is an acute exacerbation of idiopathic pulmonary fibrosis. In this chapter we review the differential diagnosis for patients with idiopathic pulmonary fibrosis who present with acute respiratory failure. Furthermore, we review the available evidence pertinent to the intensivist when these patients are cared for in the intensive care unit. Lastly, we explore treatment controversies (Extracorporeal Life Support, corticosteroids, non-invasive ventilation strategies) that are employed by some centers, but with limited evidence.