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Acute Exacerbation of Idiopathic Pulmonary Fibrosis: A Proposal

Kerri Johannson1, Harold R Collard2

  • 1Division 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.

Current Respiratory Care Reports
|January 14, 2014
PubMed

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.

Keywords:
Acute exacerbationDefinitionDiagnosisIdiopathic Pulmonary FibrosisInterstitial Lung DiseaseManagement

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