May 4, 2017

New research provides insight into rare and fatal lung disease

Recent research into Idiopathic Pulmonary Fibrosis (IPF), a rare devastating and incurable lung disease, has provided greater insight into the natural history and clinical management of the disease across Australia.

The study Baseline characteristics of Idiopathic Pulmonary Fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry, utilised data from the Lung Foundation Australia IPF Registry, which is the only national IPF registry.  Data from as many as 647 Registry participants was used to research baseline characteristics of IPF, including comorbidities, diagnostic procedures and quality of life.

Dr Helen Jo, author of the research, said the study reveals patients with the disease in Australia are not homogenous and they have a wide array of comorbidities and disease severity.

“The study shows that there are a lot of factors that can impact the patient’s prognosis including not only their age, gender and lung function, but also their symptom scores,” Dr Jo said.

“While the registry was not specifically designed to assess treatment, we also show that patients who are on antifibrotic therapy appear to have better outcomes,” she said.

Chair of the Lung Foundation Australian IPF Registry, Associate Professor Tamera Corte said the Registry, which was established in 2012, provides vital information to help researchers better understand this serious and complex disease.

“Information has not previously been gathered on the history, clinical characteristics and treatment of this rare disease in Australia,” Associate Professor Tamera Corte said.

“Unlike clinical trial cohorts were patients are carefully selected, the Registry follows any consenting patient diagnosed with IPF by their treating physician. The Registry is therefore able to describe ‘real-world’ management of IPF patients who have a diverse range of disease severity, age and co-morbidities.

“As the landscape of IPF changes with new medications becoming available to slow disease progression, the Registry will be in a unique position to examine the adoption and outcomes of new IPF management strategies,” she said.

Chief Executive Officer of Lung Foundation Australia, Ms Heather Allan, said the Registry and its research are increasing our knowledge about this rare disease, and with improved understanding we have the potential to improve health outcomes for those living with IPF.

“An estimated 1.25–63 out of 100 000 people have IPF. While it is seen as a rare disease and little is known about its natural history, its prevalence is increasing globally,” Ms Allan said.

“Lung Foundation Australia is committed to building further upon our research platforms, such as the Australian IPF Registry, to provide researchers with the information required to gain a greater understanding, earlier diagnosis and new treatment options for rare lung diseases.

“Unfortunately, lung disease struggles to attract the same level of research funding received by other disease areas.  As the only national charity dedicated to anyone with a lung disease in Australia, we are working to fill this gap,” she said.

The study Baseline characteristics of Idiopathic Pulmonary Fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry recently received international recognition through publication in the European Respiratory Journal.

To support further research into IPF, donate to Lung Foundation Australia and help spread the word about their IPF Registry. For more information visit: