title: Acute Exacerbation of Idiopathic Pulmonary Fibrosis: A Proposal.
abstract: Acute exacerbation of idiopathic pulmonary fibrosis (IPF) occurs in roughly 10% of patients annually, and is a leading cause of morbidity and mortality in this disease. While currently defined as idiopathic acute worsenings, acute exacerbations of IPF may in fact have a variety of causes, in particular infection and aspiration. Central to the pathobiology of clinically meaningful events is a diffuse injury to the IPF lung manifest histopathologically as diffuse alveolar damage, and biologically as accelerated alveolar epithelial cell injury or repair. Based on these recent observations, we propose a new paradigm for acute exacerbation of IPF that removes the idiopathic requirement and focuses on the pathophysiological mechanism involved.
authors.affiliation: Division of Respirology, Department of Medicine, University of Calgary, Calgary AB ; Division of Pulmonary, Critical Care Medicine, Sleep and Allergy, Department of Medicine, University of California, San Francisco, CA.
authors.forename: Kerri
authors.initials: K
authors.lastname: Johannson
authors.affiliation: Division of Pulmonary, Critical Care Medicine, Sleep and Allergy, Department of Medicine, University of California, San Francisco, CA.
authors.forename: Harold R
authors.initials: HR
authors.lastname: Collard
keywords: Acute exacerbation; Definition; Diagnosis; Idiopathic Pulmonary Fibrosis; Interstitial Lung Disease; Management