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Published in: Current Pulmonology Reports 4/2013

01-12-2013 | Interstitial Lung Disease (G Tino, Section editor)

Acute exacerbation of idiopathic pulmonary fibrosis: a proposal

Authors: Kerri A. Johannson, Harold R. Collard

Published in: Current Pulmonology Reports | Issue 4/2013

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Abstract

Acute exacerbation of idiopathic pulmonary fibrosis (IPF) occurs in approximately 10 % of IPF patients annually, and is a leading cause of morbidity and mortality in this disease. Although currently defined as idiopathic acute worsening, acute exacerbation 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. On the basis of these recent observations, we propose a new conceptual framework for acute exacerbation of IPF that removes the idiopathic requirement and focuses on the pathophysiological mechanism involved.
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Metadata
Title
Acute exacerbation of idiopathic pulmonary fibrosis: a proposal
Authors
Kerri A. Johannson
Harold R. Collard
Publication date
01-12-2013
Publisher
Springer US
Published in
Current Pulmonology Reports / Issue 4/2013
Electronic ISSN: 2199-2428
DOI
https://doi.org/10.1007/s13665-013-0065-x

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