Baseline characteristics of idiopathic pulmonary fibrosis

Analysis from the Australian Idiopathic Pulmonary Fibrosis Registry

Helen E. Jo, Ian Glaspole, Christopher Grainge, Nicole Goh, Peter M.A. Hopkins, Yuben Moodley, Paul N. Reynolds, Sally Chapman, E. Haydn Walters, Christopher Zappala, Heather Allan, Gregory J. Keir, Andrew Hayen, Wendy A. Cooper, Annabelle M. Mahar, Samantha Ellis, Sacha Macansh, Tamera J. Corte

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Abstract

The prevalence of idiopathic pulmonary fibrosis (IPF), a fatal and progressive lung disease, is estimated at 1.25-63 out of 100 000, making large population studies difficult. Recently, the need for large longitudinal registries to study IPF has been recognised. The Australian IPF Registry (AIPFR) is a national registry collating comprehensive longitudinal data of IPF patients across Australia. We explored the characteristics of this IPF cohort and the effect of demographic and physiological parameters and specific management on mortality. Participants in the AIPFR (n=647, mean age 70.9±8.5 years, 67.7% male, median follow up 2 years, range 6 months-4.5 years) displayed a wide range of age, disease severity and co-morbidities that is not present in clinical trial cohorts. The cumulative mortality rate in year one, two, three and four was 5%, 24%, 37% and 44% respectively. Baseline lung function (forced vital capacity, diffusing capacity of the lung for carbon monoxide, composite physiological index) and GAP (gender, age, physiology) stage (hazard ratio 4.64, 95% CI 3.33-6.47, p<0.001) were strong predictors of mortality. Patients receiving anti-fibrotic medications had better survival (hazard ratio 0.56, 95% CI 0.34-0.92, p=0.022) than those not on anti-fibrotic medications, independent of underlying disease severity. The AIPFR provides important insights into the understanding of the natural history and clinical management of IPF.

Original languageEnglish
Article number1601592
Number of pages11
JournalEuropean Respiratory Journal
Volume49
Issue number2
DOIs
Publication statusPublished - 1 Feb 2017

Cite this

Jo, Helen E. ; Glaspole, Ian ; Grainge, Christopher ; Goh, Nicole ; Hopkins, Peter M.A. ; Moodley, Yuben ; Reynolds, Paul N. ; Chapman, Sally ; Walters, E. Haydn ; Zappala, Christopher ; Allan, Heather ; Keir, Gregory J. ; Hayen, Andrew ; Cooper, Wendy A. ; Mahar, Annabelle M. ; Ellis, Samantha ; Macansh, Sacha ; Corte, Tamera J. / Baseline characteristics of idiopathic pulmonary fibrosis : Analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. In: European Respiratory Journal. 2017 ; Vol. 49, No. 2.
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title = "Baseline characteristics of idiopathic pulmonary fibrosis: Analysis from the Australian Idiopathic Pulmonary Fibrosis Registry",
abstract = "The prevalence of idiopathic pulmonary fibrosis (IPF), a fatal and progressive lung disease, is estimated at 1.25-63 out of 100 000, making large population studies difficult. Recently, the need for large longitudinal registries to study IPF has been recognised. The Australian IPF Registry (AIPFR) is a national registry collating comprehensive longitudinal data of IPF patients across Australia. We explored the characteristics of this IPF cohort and the effect of demographic and physiological parameters and specific management on mortality. Participants in the AIPFR (n=647, mean age 70.9±8.5 years, 67.7{\%} male, median follow up 2 years, range 6 months-4.5 years) displayed a wide range of age, disease severity and co-morbidities that is not present in clinical trial cohorts. The cumulative mortality rate in year one, two, three and four was 5{\%}, 24{\%}, 37{\%} and 44{\%} respectively. Baseline lung function (forced vital capacity, diffusing capacity of the lung for carbon monoxide, composite physiological index) and GAP (gender, age, physiology) stage (hazard ratio 4.64, 95{\%} CI 3.33-6.47, p<0.001) were strong predictors of mortality. Patients receiving anti-fibrotic medications had better survival (hazard ratio 0.56, 95{\%} CI 0.34-0.92, p=0.022) than those not on anti-fibrotic medications, independent of underlying disease severity. The AIPFR provides important insights into the understanding of the natural history and clinical management of IPF.",
author = "Jo, {Helen E.} and Ian Glaspole and Christopher Grainge and Nicole Goh and Hopkins, {Peter M.A.} and Yuben Moodley and Reynolds, {Paul N.} and Sally Chapman and Walters, {E. Haydn} and Christopher Zappala and Heather Allan and Keir, {Gregory J.} and Andrew Hayen and Cooper, {Wendy A.} and Mahar, {Annabelle M.} and Samantha Ellis and Sacha Macansh and Corte, {Tamera J.}",
year = "2017",
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Jo, HE, Glaspole, I, Grainge, C, Goh, N, Hopkins, PMA, Moodley, Y, Reynolds, PN, Chapman, S, Walters, EH, Zappala, C, Allan, H, Keir, GJ, Hayen, A, Cooper, WA, Mahar, AM, Ellis, S, Macansh, S & Corte, TJ 2017, 'Baseline characteristics of idiopathic pulmonary fibrosis: Analysis from the Australian Idiopathic Pulmonary Fibrosis Registry', European Respiratory Journal, vol. 49, no. 2, 1601592. https://doi.org/10.1183/13993003.01592-2016

Baseline characteristics of idiopathic pulmonary fibrosis : Analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. / Jo, Helen E.; Glaspole, Ian; Grainge, Christopher; Goh, Nicole; Hopkins, Peter M.A.; Moodley, Yuben; Reynolds, Paul N.; Chapman, Sally; Walters, E. Haydn; Zappala, Christopher; Allan, Heather; Keir, Gregory J.; Hayen, Andrew; Cooper, Wendy A.; Mahar, Annabelle M.; Ellis, Samantha; Macansh, Sacha; Corte, Tamera J.

In: European Respiratory Journal, Vol. 49, No. 2, 1601592, 01.02.2017.

Research output: Contribution to journalArticleResearchpeer-review

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T2 - Analysis from the Australian Idiopathic Pulmonary Fibrosis Registry

AU - Jo, Helen E.

AU - Glaspole, Ian

AU - Grainge, Christopher

AU - Goh, Nicole

AU - Hopkins, Peter M.A.

AU - Moodley, Yuben

AU - Reynolds, Paul N.

AU - Chapman, Sally

AU - Walters, E. Haydn

AU - Zappala, Christopher

AU - Allan, Heather

AU - Keir, Gregory J.

AU - Hayen, Andrew

AU - Cooper, Wendy A.

AU - Mahar, Annabelle M.

AU - Ellis, Samantha

AU - Macansh, Sacha

AU - Corte, Tamera J.

PY - 2017/2/1

Y1 - 2017/2/1

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