Respiratory Failure in a Patient with Idiopathic Pulmonary Fibrosis

Kumar, Anupam; Hadley, Ryan

The triggers as well as etiologies for Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) are not known. AE-IPF is defined as an “acute, clinically significant respiratory deterioration characterized by evidence of new widespread alveolar abnormality typically less than 1 month’s duration. The underlying pathologic insult is classically described as diffuse alveolar damage. Ideally, infection is excluded by BAL as in the case presentation, but the severity of hypoxemia and the desire to avoid endotracheal intubation may preclude the performance of this procedure. Supportive care is the mainstay of therapy as there are no proven therapies, although corticosteroids, cytotoxic agents and anti-coagulation have all been suggested as possible treatments. The mortality is high, particularly once invasive ventilation has been instituted.