Baseline characteristics of idiopathic pulmonary fibrosis: Analysis from the Australian Idiopathic Pulmonary Fibrosis Registry

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

Baseline characteristics of idiopathic pulmonary fibrosis: Analysis from the Australian Idiopathic Pulmonary Fibrosis Registry

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

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Publication Type:: Journal Article
Citation:: European Respiratory Journal, 2017, 49 (2)
Issue Date:: 2017-02-01

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Field	Value	Language
dc.contributor.author	Jo, HE	en_US
dc.contributor.author	Glaspole, I	en_US
dc.contributor.author	Grainge, C	en_US
dc.contributor.author	Goh, N	en_US
dc.contributor.author	Hopkins, PMA	en_US
dc.contributor.author	Moodley, Y	en_US
dc.contributor.author	Reynolds, PN	en_US
dc.contributor.author	Chapman, S	en_US
dc.contributor.author	Walters, EH	en_US
dc.contributor.author	Zappala, C	en_US
dc.contributor.author	Allan, H	en_US
dc.contributor.author	Keir, GJ	en_US
dc.contributor.author	Hayen, A https://orcid.org/0000-0003-4046-8030	en_US
dc.contributor.author	Cooper, WA	en_US
dc.contributor.author	Mahar, AM	en_US
dc.contributor.author	Ellis, S	en_US
dc.contributor.author	Macansh, S	en_US
dc.contributor.author	Corte, TJ	en_US
dc.date.available	2016-10-27	en_US
dc.date.issued	2017-02-01	en_US
dc.identifier.citation	European Respiratory Journal, 2017, 49 (2)	en_US
dc.identifier.issn	0903-1936	en_US
dc.identifier.uri	http://hdl.handle.net/10453/127515
dc.description.abstract	© Copyright ERS 2017. 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.	en_US
dc.relation.ispartof	European Respiratory Journal	en_US
dc.relation.isbasedon	10.1183/13993003.01592-2016	en_US
dc.subject.classification	Respiratory System	en_US
dc.subject.mesh	Lung	en_US
dc.subject.mesh	Humans	en_US
dc.subject.mesh	Carbon Monoxide	en_US
dc.subject.mesh	Vital Capacity	en_US
dc.subject.mesh	Registries	en_US
dc.subject.mesh	Multivariate Analysis	en_US
dc.subject.mesh	Survival Analysis	en_US
dc.subject.mesh	Follow-Up Studies	en_US
dc.subject.mesh	Prospective Studies	en_US
dc.subject.mesh	Age Distribution	en_US
dc.subject.mesh	Adult	en_US
dc.subject.mesh	Aged	en_US
dc.subject.mesh	Aged, 80 and over	en_US
dc.subject.mesh	Middle Aged	en_US
dc.subject.mesh	Australia	en_US
dc.subject.mesh	Female	en_US
dc.subject.mesh	Male	en_US
dc.subject.mesh	Idiopathic Pulmonary Fibrosis	en_US
dc.title	Baseline characteristics of idiopathic pulmonary fibrosis: Analysis from the Australian Idiopathic Pulmonary Fibrosis Registry	en_US
dc.type	Journal Article
utslib.citation.volume	2	en_US
utslib.citation.volume	49	en_US
utslib.for	0104 Statistics	en_US
utslib.for	1102 Cardiorespiratory Medicine and Haematology	en_US
utslib.for	1116 Medical Physiology	en_US
utslib.for	11 Medical and Health Sciences	en_US
pubs.embargo.period	Not known	en_US
pubs.organisational-group	/University of Technology Sydney
pubs.organisational-group	/University of Technology Sydney/Faculty of Health
pubs.organisational-group	/University of Technology Sydney/Faculty of Health/Public Health
utslib.copyright.status	closed_access
pubs.issue	2	en_US
pubs.publication-status	Published	en_US
pubs.volume	49	en_US

Abstract:

© Copyright ERS 2017. 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.

Please use this identifier to cite or link to this item:

http://hdl.handle.net/10453/127515