RT Journal Article
SR Electronic
T1 Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1601592
DO 10.1183/13993003.01592-2016
VO 49
IS 2
A1 Helen E. Jo
A1 Ian Glaspole
A1 Christopher Grainge
A1 Nicole Goh
A1 Peter M.A. Hopkins
A1 Yuben Moodley
A1 Paul N. Reynolds
A1 Sally Chapman
A1 E. Haydn Walters
A1 Christopher Zappala
A1 Heather Allan
A1 Gregory J. Keir
A1 Andrew Hayen
A1 Wendy A. Cooper
A1 Annabelle M. Mahar
A1 Samantha Ellis
A1 Sacha Macansh
A1 Tamera J. Corte
YR 2017
UL http://erj.ersjournals.com/content/49/2/1601592.abstract
AB 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.Data from the Australian IPF registry shows anti-fibrotic therapy and baseline physiology predict survival in IPF http://ow.ly/Ete2305OkU9