| Authors | Jo, H.E. Douglass, J.A. Grainge, C. Goh, N. Hopkins, P.M. 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. |
|---|---|
| Type | Journal Article (Original Research) |
| Journal | European Respiratory Journal |
| PubMed ID | 28232409 |
| Year of Publication | 2018 |
| URL | https://www.ncbi.nlm.nih.gov/pubmed/28232409 |
| Download |  1601592full_.pdf (522.5 KB) |
| 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. |
http://www.ibas.org.au/what-we-do/publications/3872944
Motor neurone disease (MND) causes the body's muscles to weaken. Breathing muscle weakness means that most people affected by MND will eventually lose the ability to take a deep breath and cough strongly....
RESPIRATORY BIOMARKERS IN MOTOR NEURONE DISEASE
The inability to breathe is unfortunately the most common cause of death in people living with Motor Neurone Disease (MND). Last year, our clinical research group in Melbourne reported that breathing...
Sleep apnea is a condition where breathing is abnormal during sleep. There are two main forms of sleep apnea: obstructive and central. For obstructive sleep apnea, breathing is reduced because the airway...
PERTH HIGHLIGHTS: TSANZSRS 2026
A huge congratulations to all our team members who participated in the excellent workshops and meetings at the TSANZSRS Annual Scientific Meeting in Perth in March 2026!
BETTER SLEEP, BETTER LIFE TIPS
For World Sleep Day, Dr. Amy Jordan joined the "Get Cereal" panelists Sam and Matilda to share top tips for better sleep, discussing the vital importance of sleep health and hygiene in a podcast interview.
Kudos to Dr. Lauren Booker & Dr. Jen Cori on their JOEM publication examining fatigue detection alarms in rural truck drivers. Their study explores the alarms' effectiveness, accuracy, and habituation, offering key insights into fatigue management.
HONORING EXCELLENCE IN RESEARCH
Congratulations to Prof. Anne Holland and A/Prof. Narelle Cox for being featured in the NHMRC's 10 of the Best - 16th Edition. Their work exemplifies groundbreaking research delivering extraordinary outcomes.
© 2026 Institute for Breathing and Sleep. All Rights Reserved. ABN 39 093 685 879
Powered by Helmut Design | Manage
