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 amount of scar tissue irreversibly increases over time. The rate at which the disease progresses is highly variable, with some patients remaining stable for many years while others may rapidly get worse.

IPF often remains undiagnosed because the symptoms are similar to other lung diseases such as severe asthma, chronic obstructive pulmonary disease, congestive heart failure and other treatable forms of lung fibrosis.

These symptoms include:

  • Shortness of breath on exertion
  • 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. However, it is known that the disease is more common in smokers. 70% of those diagnosed have a history of significant nicotine consumption.

It is estimated that about 5,000 people have IPF in Australia. However, due to the idiopathic nature of the disease, it is difficult to collate exact numbers.

IPF Fact Sheet:

Idiopathic Pulmonary Fibrosis_FS Dec15 (533 KB)

Idiopathic Pulmonary Fibrosis Peer Support

A model of patient support where patients living with IPF connect via phone with other IPF Patients.

Be part of a support group that gives patients the unique opportunity to:

  • Share experiences and knowledge
  • Connect with other IPF patients
  • Offer support to one another over the phone
  • Access emotional support and information

For more information please contact our Information and Support Centre on 1800 654 301 or email

Australian IPF Registry

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 IVRecruiting now
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
WASvetlana Baltic08 6151 0890 Email Svetlana
NTSacha Macansh02 9515 3996Email Sacha