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

H.E. Jo, I. Glaspole, Christopher Grainge, N. Goh, P. M. A. Hopkins, Y. Moodley, P.N Reynolds, S. Chapman, E. H. Walters, C. Zappala, H. Allan, G.J. Keir, A. Hayen, W. A. Cooper, A.M. Mahar, S. Ellis, S. Macansh, T.J. Corte

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Abstract

7The 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
JournalThe European Respiratory Journal
Volume49
Issue number2
DOIs
Publication statusPublished - Feb 2017
Externally publishedYes

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Idiopathic Pulmonary Fibrosis
Registries
Mortality
Lung Volume Measurements
Cohort Effect
Vital Capacity
Carbon Monoxide
Natural History
Lung Diseases
Demography
Clinical Trials
Morbidity
Lung
Survival
Population

Cite this

Jo, H.E. ; Glaspole, I. ; Grainge, Christopher ; Goh, N. ; Hopkins, P. M. A. ; Moodley, Y. ; Reynolds, P.N ; Chapman, S. ; Walters, E. H. ; Zappala, C. ; Allan, H. ; Keir, G.J. ; Hayen, A. ; Cooper, W. A. ; Mahar, A.M. ; Ellis, S. ; Macansh, S. ; Corte, T.J. / Baseline characteristics of idiopathic pulmonary fibrosis : analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. In: The 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 = "7The 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.",
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author = "H.E. Jo and I. Glaspole and Christopher Grainge and N. Goh and Hopkins, {P. M. A.} and Y. Moodley and P.N Reynolds and S. Chapman and Walters, {E. H.} and C. Zappala and H. Allan and G.J. Keir and A. Hayen and Cooper, {W. A.} and A.M. Mahar and S. Ellis and S. Macansh and T.J. Corte",
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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' The 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, H.E.; Glaspole, I.; Grainge, Christopher; Goh, N.; Hopkins, P. M. A.; Moodley, Y.; Reynolds, P.N; Chapman, S.; Walters, E. H.; Zappala, C.; Allan, H.; Keir, G.J.; Hayen, A.; Cooper, W. A.; Mahar, A.M.; Ellis, S.; Macansh, S.; Corte, T.J.

In: The European Respiratory Journal, Vol. 49, No. 2, 1601592, 02.2017.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Baseline characteristics of idiopathic pulmonary fibrosis

T2 - analysis from the Australian Idiopathic Pulmonary Fibrosis Registry

AU - Jo, H.E.

AU - Glaspole, I.

AU - Grainge, Christopher

AU - Goh, N.

AU - Hopkins, P. M. A.

AU - Moodley, Y.

AU - Reynolds, P.N

AU - Chapman, S.

AU - Walters, E. H.

AU - Zappala, C.

AU - Allan, H.

AU - Keir, G.J.

AU - Hayen, A.

AU - Cooper, W. A.

AU - Mahar, A.M.

AU - Ellis, S.

AU - Macansh, S.

AU - Corte, T.J.

N1 - Copyright ©ERS 2017.

PY - 2017/2

Y1 - 2017/2

N2 - 7The 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.

AB - 7The 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.

KW - Adult

KW - Age Distribution

KW - Aged

KW - Aged, 80 and over

KW - Australia/epidemiology

KW - Carbon Monoxide/blood

KW - Female

KW - Follow-Up Studies

KW - Humans

KW - Idiopathic Pulmonary Fibrosis/drug therapy

KW - Lung/physiopathology

KW - Male

KW - Middle Aged

KW - Multivariate Analysis

KW - Prospective Studies

KW - Registries

KW - Survival Analysis

KW - Vital Capacity

U2 - 10.1183/13993003.01592-2016

DO - 10.1183/13993003.01592-2016

M3 - Article

VL - 49

JO - The European Respiratory Journal

JF - The European Respiratory Journal

SN - 0903-1936

IS - 2

M1 - 1601592

ER -