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Title: Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry.
Austin Authors: Jo, Helen E;Glaspole, Ian;Grainge, Christopher;Goh, Nicole S L ;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
Affiliation: Dept of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, Australia
Faculty of Medicine, University of Sydney, Sydney, Australia
Dept of Allergy and Respiratory Medicine, The Alfred Hospital, Melbourne, Australia
Faculty of Medicine, Monash University, Melbourne, Australia
Dept of Respiratory Medicine, John Hunter Hospital, Newcastle, Australia
Department of Respiratory and Sleep Medicine, Austin Health, Heidelberg, Victoria, Australia
School of Medicine, University of Queensland, Brisbane, Australia
Dept of Respiratory Medicine, Fiona Stanley Hospital, Perth, Australia
Dept of Respiratory Medicine, Royal Adelaide Hospital, Adelaide, Australia
University of Tasmania, Hobart, Australia
Dept of Thoracic Medicine, Royal Brisbane & Women's Hospital, Brisbane, Australia
Lung Foundation Australia, Brisbane, Australia
Dept of Respiratory Medicine, Princess Alexandra Hospital, Brisbane, Australia
Tissue Pathology and Diagnostic Oncology, Royal Prince Alfred Hospital, Sydney, Australia
Dept of Radiology, The Alfred Hospital, Melbourne, Australia
Issue Date: Feb-2017 2017-02-23
Publication information: The European respiratory journal 2017; 49(2): 1601592
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.
DOI: 10.1183/13993003.01592-2016
ORCID: 0000-0003-1183-2729
PubMed URL: 28232409
Type: Journal Article
Appears in Collections:Journal articles

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