Skip to main content

Main menu

  • Home
  • Current issue
  • Early View
  • Archive
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • COVID-19 submission information
    • Institutional open access agreements
    • Peer reviewer login
  • Alerts
  • Subscriptions
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

User menu

  • Log in
  • Subscribe
  • Contact Us
  • My Cart

Search

  • Advanced search
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

Login

European Respiratory Society

Advanced Search

  • Home
  • Current issue
  • Early View
  • Archive
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • COVID-19 submission information
    • Institutional open access agreements
    • Peer reviewer login
  • Alerts
  • Subscriptions

Mortality and survival in idiopathic pulmonary fibrosis: a systematic review and meta-analysis

Qiang Zheng, Ingrid A. Cox, Julie A. Campbell, Qing Xia, Petr Otahal, Barbara de Graaff, Tamera J. Corte, Alan K. Y. Teoh, E. Haydn Walters, Andrew J. Palmer
ERJ Open Research 2022; DOI: 10.1183/23120541.00591-2021
Qiang Zheng
1Menzies Institute for Medical Research, University of Tasmania, Hobart, Tasmania, Australia
2NHMRC Centre of Research Excellence for Pulmonary Fibrosis, Camperdown, Australia
3Dept of Anesthesiology (High-Tech Branch), the First Affiliated Hospital of Anhui Medical University, Hefei, Anhui, China
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Qiang Zheng
Ingrid A. Cox
1Menzies Institute for Medical Research, University of Tasmania, Hobart, Tasmania, Australia
2NHMRC Centre of Research Excellence for Pulmonary Fibrosis, Camperdown, Australia
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Ingrid A. Cox
Julie A. Campbell
1Menzies Institute for Medical Research, University of Tasmania, Hobart, Tasmania, Australia
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Qing Xia
1Menzies Institute for Medical Research, University of Tasmania, Hobart, Tasmania, Australia
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Petr Otahal
1Menzies Institute for Medical Research, University of Tasmania, Hobart, Tasmania, Australia
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Barbara de Graaff
1Menzies Institute for Medical Research, University of Tasmania, Hobart, Tasmania, Australia
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Tamera J. Corte
2NHMRC Centre of Research Excellence for Pulmonary Fibrosis, Camperdown, Australia
4Central Clinical School, The University of Sydney, Camperdown, Australia
5Dept of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Camperdown, Australia
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Alan K. Y. Teoh
2NHMRC Centre of Research Excellence for Pulmonary Fibrosis, Camperdown, Australia
4Central Clinical School, The University of Sydney, Camperdown, Australia
5Dept of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Camperdown, Australia
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
E. Haydn Walters
6School of Medicine, University of Tasmania, Hobart, Tasmania, Australia
7School of Population and Global Health, University of Melbourne, Melbourne, Victoria, Australia
8Joint senior authors
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Andrew J. Palmer
1Menzies Institute for Medical Research, University of Tasmania, Hobart, Tasmania, Australia
2NHMRC Centre of Research Excellence for Pulmonary Fibrosis, Camperdown, Australia
7School of Population and Global Health, University of Melbourne, Melbourne, Victoria, Australia
8Joint senior authors
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • For correspondence: andrew.palmer@utas.edu.au
  • Article
  • Info & Metrics
  • PDF
Loading

Abstract

Background : There are substantial advances in diagnosis and treatment for idiopathic pulmonary fibrosis (IPF), but without much evidence available on recent mortality and survival trends.

Methods : A narrative synthesis approach was used to investigate the mortality trends, then meta-analyses for survival trends were carried out based on various time periods.

Results : Six studies reported the mortality data for IPF in 22 countries, and 62 studies (covering 63 307 patients from 20 countries) reported survival data for IPF. Age-standardised mortality for IPF varied from approximately 0.5 to 12 per 100 000 population per year after year 2000. There were increased mortality trends for IPF in Australia, Brazil, Belgium, Canada, Czech Republic, Finland, France, Germany, Hungary, Italy, Lithuania, Netherlands, Poland, Portugal, Spain, Sweden, and UK, while Austria, Croatia, Denmark, Romania, and US showed decreased mortality trends. The overall 3-year and 5-year cumulative survival rates (CSRs) were 61.8% (95% CI, 58.7–64.9; I2=97.1%) and 45.6% (95% CI, 41.5–49.7; I2=97.7%), respectively. Prior to 2010, the pooled 3-year CSRs was 59.9% (95% CI, 55.8–64.1; I2>95.8%), then not significantly (p=0.067) increased to 66.2% (95% CI, 62.9–69.5; I2=92.6%) in the 2010s decade. After excluding three studies in which no patients received antifibrotics after year 2010, the pooled 3-year CSRs significantly (p=0.039) increased to 67.4% (95% CI, 63.9–70.9; I2=93.1%) in the 2010s decade.

Discussion : IPF is a diagnosis associated with high mortality. There was no observed increasing survival trend for patients with IPF before year 2010, with then a switch to an improvement, which is probably multifactorial.

Footnotes

This manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.

Conflict of interest: Qiang Zheng has nothing to disclose.

Conflict of interest: Ingrid A. Cox has nothing to disclose.

Conflict of interest: Julie A. Campbell has nothing to disclose.

Conflict of interest: Qing Xia has nothing to disclose.

Conflict of interest: Petr Otahal has nothing to disclose.

Conflict of interest: Barbara de Graaff has nothing to disclose.

Conflict of interest: Tamera J. Corte reports grants, personal fees, and non-financial support from Boehringer Ingelheim, grants, personal fees and non-financial support from Hoffman La Roche, grants and personal fees from Bristol Myers Squibb, grants from Avalyn Pharma, grants from Biogen, personal fees from Promedior, from null, outside the submitted work

Conflict of interest: Alan K Y Teoh reports conference fees from Boehringer Ingelheim and speaker fees from Roche, outside the submitted work.

Conflict of interest: E. Haydn Walters has nothing to disclose.

Conflict of interest: Andrew J. Palmer has nothing to disclose.

This is a PDF-only article. Please click on the PDF link above to read it.

  • Received October 19, 2021.
  • Accepted January 9, 2022.
  • Copyright ©The authors 2022
http://creativecommons.org/licenses/by-nc/4.0/

This version is distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0. For commercial reproduction rights and permissions contact permissions{at}ersnet.org

PreviousNext
Back to top
Vol 8 Issue 2 Table of Contents
ERJ Open Research: 8 (2)
  • Table of Contents
  • Index by author
Email

Thank you for your interest in spreading the word on European Respiratory Society .

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Mortality and survival in idiopathic pulmonary fibrosis: a systematic review and meta-analysis
(Your Name) has sent you a message from European Respiratory Society
(Your Name) thought you would like to see the European Respiratory Society web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Print
Citation Tools
Mortality and survival in idiopathic pulmonary fibrosis: a systematic review and meta-analysis
Qiang Zheng, Ingrid A. Cox, Julie A. Campbell, Qing Xia, Petr Otahal, Barbara de Graaff, Tamera J. Corte, Alan K. Y. Teoh, E. Haydn Walters, Andrew J. Palmer
ERJ Open Research Jan 2022, 00591-2021; DOI: 10.1183/23120541.00591-2021

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Share
Mortality and survival in idiopathic pulmonary fibrosis: a systematic review and meta-analysis
Qiang Zheng, Ingrid A. Cox, Julie A. Campbell, Qing Xia, Petr Otahal, Barbara de Graaff, Tamera J. Corte, Alan K. Y. Teoh, E. Haydn Walters, Andrew J. Palmer
ERJ Open Research Jan 2022, 00591-2021; DOI: 10.1183/23120541.00591-2021
del.icio.us logo Digg logo Reddit logo Technorati logo Twitter logo CiteULike logo Connotea logo Facebook logo Google logo Mendeley logo
Full Text (PDF)

Jump To

  • Article
    • Abstract
  • Info & Metrics
  • PDF

Subjects

  • Interstitial and orphan lung disease
  • Tweet Widget
  • Facebook Like
  • Google Plus One

More in this TOC Section

  • A Systematic Review with Meta-analysis of Biomarkers for detection of Pulmonary Arterial Hypertension
  • Recurrence of primary disease following lung transplantation
  • Pulmonary Function Test and CT features during follow-up after SARS, MERS and COVID-19: A Systematic Review and Meta-Analysis
Show more Review

Related Articles

Navigate

  • Home
  • Current issue
  • Archive

About ERJ Open Research

  • Editorial board
  • Journal information
  • Press
  • Permissions and reprints
  • Advertising

The European Respiratory Society

  • Society home
  • myERS
  • Privacy policy
  • Accessibility

ERS publications

  • European Respiratory Journal
  • ERJ Open Research
  • European Respiratory Review
  • Breathe
  • ERS books online
  • ERS Bookshop

Help

  • Feedback

For authors

  • Instructions for authors
  • Publication ethics and malpractice
  • Submit a manuscript

For readers

  • Alerts
  • Subjects
  • RSS

Subscriptions

  • Accessing the ERS publications

Contact us

European Respiratory Society
442 Glossop Road
Sheffield S10 2PX
United Kingdom
Tel: +44 114 2672860
Email: journals@ersnet.org

ISSN

Online ISSN: 2312-0541

Copyright © 2022 by the European Respiratory Society