Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic Pulmonary Fibrosis (IPF) is a condition that causes persistent and progressive scarring of the tiny air sacs (alveoli) in the lungs. The alveoli perform the vital functions of transferring oxygen to your blood stream from the air you breathe in, and transferring the waste product, carbon dioxide from your blood, to the air you breathe out. The amount of scar tissue irreversibly increases over time. This is a serious condition, however the rate at which the disease progresses is variable, with some patients remaining stable for years while others may deteriorate rapidly.

The symptoms of Idiopathic Pulmonary Fibrosis include:

  • Shortness of breath on exertion – particularly noticeable when walking up hills or stairs
  • Cough (generally dry)
  • Fingernails and toenails change to a beak shape, known as clubbing
At present, it is not known what causes the disease to develop and progress. The term ‘idiopathic’ literally means ‘of no known cause’ although it is known that the disease is more common in smokers (70% of those diagnosed have a history of significant nicotine consumption). As a general rule, IPF is not passed on to siblings or children, but on occasions, several members of a family may be affected. This suggests that one’s genetic profile may be a factor in the cause of the illness.
We don’t really know how many people in Australia have IPF, but based on international rates, we estimate that approximately 2,300 new cases of IPF are diagnosed every year. The risk of IPF increases with age; the disease is uncommon below the age of 50 years. Men are slightly more commonly affected than women and the disease affects people of all races.
The diagnosis of IPF is challenging and requires careful evaluation of clinical, laboratory, x-ray data, high resolution computed tomography (HRCT) and sometimes lung biopsy material to make a confident diagnosis. This is usually made by a respiratory specialist in conjunction with other specialists with an interest in IPF.
You can contact the Lung Foundation Information and Support service to obtain resources about IPF and living well with lung disease. You can also be referred to a Peer support group for people living with IPF and their carers. You can also be referred to a Peer support group for people living with IPF and their carers.
Being part of a peer support group offers people impacted by IPF the unique opportunity to:

  • Share experiences and knowledge
  • Connect with other people who understand what you are going through
  • Offer support to one another over the phone
  • Access general support and information

To be referred to the Pulmonary Fibrosis Peer Support please contact our Information and Support Centre on 1800 654 301 or email

You can download our IPF factsheet here:

Idiopathic Pulmonary Fibrosis_FS Dec15 (533 KB)

Lung Foundation Australia has developed the Australian IPF Registry to enable us to collect information about the numbers of people with IPF in Australia.

The information collected by the registry may potentially be used to address many different research questions across epidemiology, medical and drug trial research.

The Australian IPF Registry provides many benefits including:

  • A unique platform for epidemiological and clinical research
  • Improved recruitment into clinical trials
  • Increased IPF disease awareness and improved tertiary referral
  • Improved patient understanding and support networks
  • Improved Australian and international IPF research collaboration
  • Expert specialists provide feedback on HRCT scans and/or surgical biopsy slides.

Clinical trials for IPF medications in Australia

A number of clinical trials in IPF are currently being or have recently been undertaken in Australia. The future of IPF therapy rests with well-designed clinical trials to evaluate the ability of new therapies to stop or reverse existing lung scarring. It is recommended to discuss the possibility of taking part in a clinical trial with your specialist. A list of the clinical trials which are being conducted.

StudyTherapeutic agent Company Trial PhaseProgress
BI 11.99.36*Sildenafil (*Nintedanib)Boehringer IngelheimPhase IIIbRecruiting now
BI 1199.227NintedanibBoehringer IngelheimPhase IVRecruitment complete
LebrikizumabRochePhase IIRecruitment complete
SAR156597SanofiPhase IIRecruitment complete
BMS-986020Bristol-Myers Squibb Phase IIRecruitment complete
NCT02510937CC-90001CelgenePhase IRecruitment complete
* Background antifibrotic medication allowed.

Table 1. Clinical trials for potential IPF medications in Australia

For further information on these trials please speak to your respiratory physician.

If you have Idiopathic Pulmonary Fibrosis you too can help, please ask your respiratory physician about the Registry during your next visit.

Health care providers please contact the Registry Coordinator in your State to inform them of your IPF patients who are interested in joining the Registry.

Registry Coordinators

NSW & ACTRobert Henson02 9515 4018Email Robert
QLDSacha Macansh02 9515 3996Email Sacha
SAAlysha Riley08 8222 4244 Email Alysha
TASHeather Chaplin03 6226 4790Email Heather
VICKaren Symons03 9076 6963Email Karen
WASacha Macansh02 9515 3996Email Sacha
NTSacha Macansh02 9515 3996Email Sacha


Bill’s story – IPF a life changer

I was originally diagnosed with potential IPF on in March 2015 when some indicators were noticed on an X-Ray and subsequent CT scan I’d had to ensure a bout of pneumonia had cleared up.

The diagnosis did still take the wind out of my sails. It also was a bit of a shock for the family, with reactions predicated by having watched my sister, their Aunt, go through something similar everal years earlier, at a time when little could be done.

Read more

COPD Action Plan and Steps for writing plan May 2015