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. 2013 Dec;2(4):10.1007/s13665-013-0065-x.
doi: 10.1007/s13665-013-0065-x.

Acute Exacerbation of Idiopathic Pulmonary Fibrosis: A Proposal

Kerri Johannson  1 , Harold R Collard  2
Affiliations

Acute Exacerbation of Idiopathic Pulmonary Fibrosis: A Proposal

Kerri Johannson et al. Curr Respir Care Rep. 2013 Dec.

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.

Keywords: Acute exacerbation; Definition; Diagnosis; Idiopathic Pulmonary Fibrosis; Interstitial Lung Disease; Management.

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Conflict of interest statement

Compliance with Ethics Guidelines

Conflict of Interest

Kerri Johannson declares that she has no conflicts of interest. Harold R. Collard is a consultant for Biogen, FibroGen, Genoa, Gilead, InterMune, MedImmune, Mesoblast, and Promedior. His institution receives money as a result. His institution also receives funding through grants he has from Boehringer-Ingelheim, Genentech, and NIH/NGLBI.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by the authors.

Figures

Figure 1
Figure 1. Proposed conceptual framework for acute exacerbation of idiopathic pulmonary fibrosis (IPF)
Patients with IPF and accelerated worsening of their dyspnea can have parenchymal or extra-parenchymal explanations for their symptoms. We propose that those with parenchymal causes be categorized as acute respiratory worsening. Cases of acute respiratory worsening that demonstrate evidence of diffuse alveolar injury on high-resolution computed tomography (HRCT) scanning are subsequently categorized as acute exacerbation of IPF. In some cases of acute exacerbation of IPF, a cause will be identified (e.g. infection). Cases of acute respiratory worsening that do not have HRCT evidence for diffuse alveolar injury are categorized as other acute respiratory worsening, possible explanations for which are bronchitis, lobar pneumonia, and sub-radiographic acute exacerbation of IPF. Abbreviations: PE = pulmonary embolism; CHF = congestive heart failure; PTX = pneumothorax; HRCT = high resolution computed tomography; IPF = idiopathic pulmonary fibrosis.
See this image and copyright information in PMC

References

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    1. Collard HR, et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2007;176(7):636–43. - PMC - PubMed
    1. Song JW, et al. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J. 2011;37(2):356–63. This manuscript represents the largest descriptive cohort to date describing clinical features and outcomes in acute exacerbations of IPF. It also demonstrates that acute exacerbation may occur after surgery. - PubMed
    1. Kondoh Y, et al. Acute exacerbation in idiopathic pulmonary fibrosis. Analysis of clinical and pathologic findings in three cases. Chest. 1993;103(6):1808–12. - PubMed
    1. Kondo ASS. Intractable Diseases Research Foundation Publication No. 27 . Interstitial pneumonia of unknown etiology. Tokyo, Japan: University of Tokyo Press; 1989. Acute exacerbation in idiopathic interstitial pneumonia (IIP)

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