Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2018 Dec 21;27(150):180071.
doi: 10.1183/16000617.0071-2018. Print 2018 Dec 31.

Acute exacerbations of progressive-fibrosing interstitial lung diseases

Martin Kolb  1 , Benjamin Bondue  2 , Alberto Pesci  3 , Yasunari Miyazaki  4 , Jin Woo Song  5 , Nitin Y Bhatt  6 , John T Huggins  7 , Justin M Oldham  8 , Maria L Padilla  9 , Jesse Roman  10 , Shane Shapera  11
Affiliations
Review

Acute exacerbations of progressive-fibrosing interstitial lung diseases

Martin Kolb et al. Eur Respir Rev. .

Abstract

Acute exacerbation of interstitial lung disease (ILD) is associated with a poor prognosis and high mortality. Numerous studies have documented acute exacerbation in idiopathic pulmonary fibrosis (IPF), but less is known about these events in other ILDs that may present a progressive-fibrosing phenotype. We propose defining acute exacerbation as an acute, clinically significant respiratory deterioration, typically less than 1 month in duration, together with computerised tomography imaging showing new bilateral glass opacity and/or consolidation superimposed on a background pattern consistent with fibrosing ILDs. Drawing on observations in IPF, it is suspected that epithelial injury or proliferation and autoimmunity are risk factors for acute exacerbation in ILDs that may present a progressive-fibrosing phenotype, but further studies are required. Current acute exacerbation management strategies are based on recommendations in IPF, but no randomised controlled trials of acute exacerbation management have been performed. Although there are no formal strategies to prevent the development of acute exacerbation, possible approaches include antifibrotic drugs (such as nintedanib and pirfenidone), and minimising exposure to infection, airborne irritants and pollutants. This review discusses the current knowledge of acute exacerbation of ILDs that may present a progressive-fibrosing phenotype and acknowledges limitations of the data available.

PubMed Disclaimer

Conflict of interest statement

Conflict of interest: M. Kolb reports grants and personal fees from Roche, Boehringer Ingelheim and Prometic, personal fees from Gilead and Genoa, and grants from Actelion, Respivert, Alkermes and Pharmaxis, outside the submitted work. Conflict of interest: B. Bondue reports grants and personal fees from Boehringer Ingelheim and Le Roche-Hoffmann-La Roche, outside the submitted work. Conflict of interest: A. Pesci has nothing to disclose. Conflict of interest: Y. Miyazaki reports grants and personal fees from Nippon Boehringer Ingelheim and grants from Shionogi, outside the submitted work. Conflict of interest: J.W. Song has nothing to disclose. Conflict of interest: N.Y. Bhatt has nothing to disclose. Conflict of interest: J.T. Huggins reports grants and other fees from Roche/Genentech and Boehringer Ingelheim, during the conduct of the study. Conflict of interest: J.M. Oldham reports other support from Boehringer Ingelheim for writing fees, during the conduct of the study; and personal fees (for speakers fees and advisory board fees) from Boehringer Ingelheim and Genentech, outside the submitted work. Conflict of interest: M.L. Padilla reports personal fees from Boehringer Ingelheim and Genentech, outside the submitted work. Conflict of interest: J. Roman reports grants and personal fees from Boehringer Ingelheim and is a member of the Medical Advisory Board of Pulmonary Fibrosis Foundation. Conflict of interest: S. Shapera reports personal fees from AstraZeneca, grants, personal fees (participation in clinical trials) and other from Boehringer Ingelheim, Canada and Hoffman La-Roche, Canada, and other (participation in clinical trials) from Medimmune, ProMetic Canada and Sanofi-Aventis, outside the submitted work.

Figures

FIGURE 1
FIGURE 1
A 63-year-old woman with rheumatoid arthritis-associated interstitial lung disease who presented to clinic with 2 weeks of increasing shortness of breath. Axial and coronal images show a) baseline computed tomography 1 year before presentation showing reticulation and honeycombing in a definite usual interstitial pneumonia pattern; b) presentation of acute exacerbation of interstitial lung disease with new onset bilateral ground-glass opacities on background of usual interstitial pneumonia and c) follow-up computed tomography 1 week after treatment with high-dose steroids showing some improvement in ground-glass opacification.
See this image and copyright information in PMC

Comment in

References

    1. Flaherty KR, Brown KK, Wells AU, et al. . Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Respir Res 2017; 4: e000212. - PMC - PubMed
    1. Wells AU, Brown KK, Flaherty KR, et al. . What's in a name? That which we call IPF, by any other name would act the same. Eur Respir J 2018; 51: 1800692. - PubMed
    1. Cottin V, Hirani NA, Hotchkin DL, et al. . Presentation, diagnosis and clinical course of the spectrum of progressive fibrosing interstitial lung diseases. Eur Respir Rev 2017; 26: 180076. - PMC - PubMed
    1. Churg A, Wright JL, Tazelaar HD. Acute exacerbations of fibrotic interstitial lung disease. Histopathology 2011; 58: 525–530. - PubMed
    1. Leuschner G, Behr J. Acute exacerbation in interstitial lung disease. Front Med (Lausanne) 2017; 4: 176. - PMC - PubMed

MeSH terms