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.
authors.affiliation: Dept of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, Australia tameracorte@me.com.|Faculty of Medicine, University of Sydney, Sydney, Australia.
authors.forename: Helen E
authors.identifier: 0000-0003-1183-2729
authors.initials: HE
authors.lastname: Jo
authors.affiliation: Dept of Allergy and Respiratory Medicine, The Alfred Hospital, Melbourne, Australia.|Faculty of Medicine, Monash University, Melbourne, Australia.
authors.forename: Ian
authors.initials: I
authors.lastname: Glaspole
authors.affiliation: Dept of Respiratory Medicine, John Hunter Hospital, Newcastle, Australia.
authors.forename: Christopher
authors.initials: C
authors.lastname: Grainge
authors.affiliation: Dept of Allergy and Respiratory Medicine, The Alfred Hospital, Melbourne, Australia.|Dept of Respiratory Medicine, Austin Hospital, Heidelberg, Australia.
authors.forename: Nicole
authors.initials: N
authors.lastname: Goh
authors.affiliation: School of Medicine, University of Queensland, Brisbane, Australia.
authors.forename: Peter M A
authors.initials: PM
authors.lastname: Hopkins
authors.affiliation: Dept of Respiratory Medicine, Fiona Stanley Hospital, Perth, Australia.
authors.forename: Yuben
authors.initials: Y
authors.lastname: Moodley
authors.affiliation: Dept of Respiratory Medicine, Royal Adelaide Hospital, Adelaide, Australia.
authors.forename: Paul N
authors.initials: PN
authors.lastname: Reynolds
authors.affiliation: Dept of Respiratory Medicine, Royal Adelaide Hospital, Adelaide, Australia.
authors.forename: Sally
authors.initials: S
authors.lastname: Chapman
authors.affiliation: University of Tasmania, Hobart, Australia.
authors.forename: E Haydn
authors.initials: EH
authors.lastname: Walters
authors.affiliation: Dept of Thoracic Medicine, Royal Brisbane & Women's Hospital, Brisbane, Australia.
authors.forename: Christopher
authors.initials: C
authors.lastname: Zappala
authors.affiliation: Lung Foundation Australia, Brisbane, Australia.
authors.forename: Heather
authors.initials: H
authors.lastname: Allan
authors.affiliation: Dept of Respiratory Medicine, Princess Alexandra Hospital, Brisbane, Australia.
authors.forename: Gregory J
authors.initials: GJ
authors.lastname: Keir
authors.affiliation: Tissue Pathology and Diagnostic Oncology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Andrew
authors.initials: A
authors.lastname: Hayen
authors.affiliation: Tissue Pathology and Diagnostic Oncology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Wendy A
authors.initials: WA
authors.lastname: Cooper
authors.affiliation: Dept of Radiology, The Alfred Hospital, Melbourne, Australia.
authors.forename: Annabelle M
authors.initials: AM
authors.lastname: Mahar
authors.affiliation: Dept of Radiology, The Alfred Hospital, Melbourne, Australia.
authors.forename: Samantha
authors.initials: S
authors.lastname: Ellis
authors.affiliation: Lung Foundation Australia, Brisbane, Australia.
authors.forename: Sacha
authors.initials: S
authors.lastname: Macansh
authors.affiliation: Dept of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, Australia tameracorte@me.com.|Faculty of Medicine, University of Sydney, Sydney, Australia.
authors.forename: Tamera J
authors.initials: TJ
authors.lastname: Corte
mesh_terms: D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D001315:Australia / Q000453:epidemiology; D002248:Carbon Monoxide / Q000097:blood; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy / Q000401:mortality* / Q000503:physiopathology*; D008168:Lung / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011446:Prospective Studies; D012042:Registries; D016019:Survival Analysis; D014797:Vital Capacity
chemical_list: D002248:Carbon Monoxide