IPF (Idiopathic Pulmonary Fibrosis)

What is Idiopathic Pulmonary Fibrosis (IPF)?

Idiopathic Pulmonary Fibrosis (IPF) is a common type of Interstitial Lung Disease (ILD) that causes thickening and scarring (fibrosis) of the lung tissue.

The scarring makes it difficult for the lungs to transfer oxygen into the blood stream and deliver enough oxygen to the rest of the body.

What are the causes of IPF? 

We don’t fully understand what causes IPF. The term ‘idiopathic’ means no known cause.

However, there are some factors that can increase your risk of IPF. Common factors can include:

  • A family history of IPF called familial pulmonary fibrosis
  • Smoking or having previously smoked
  • Gastro-oesophageal reflux disease (GORD) may contribute to progression of disease.

IPF also tends to be more common in men aged over 50.

What are the signs and symptoms of IPF?

The two most common signs of IPF are:

  • A prolonged, dry cough – most people with IPF have a cough which lasts more than 8 weeks
  • Breathlessness – shortness of breath appears during exercise, and as the scarring gets worst, you may feel breathless while resting, too.

Other symptoms of IPF can include:

  • Body aches – in the muscles and joints
  • Clubbing – a widening and rounding of the fingertips and toes
  • Fatigue – feeling tired and having no energy
  • Unexplained weight loss.

What are the possible tests to diagnose IPF?

Your doctor may need to order a rage of tests before diagnosing IPF. Common tests may include:

  • Medical history – asking questions to gather information about your family history, exposure to hazardous substances and any other medical conditions
  • Physical examination – using a stethoscope to listen to your lungs
  • Imaging tests – including a Chest X-ray and CT scan to see if there is any scarring in your lungs
  • Lung function tests – to measure the size and function of your lungs
  • Bronchoscopy – using a thin tube inserted into your nose and down into your lungs to collect a small sample of fluid or lung tissue
  • Lung biopsy – Occasionally the diagnosis it not clear and a surgical procedure to sample lung tissue is needed so a pathologist can look directly at your lung tissue and help in making a diagnosis and guide treatment

As IPF is a rare disease, it is difficult to diagnose. Some doctors may not be familiar with the disease. IPF symptoms are common and present in many other types of lung disease. It can take years to get an accurate diagnosis as the condition evolves with time.

IPF worsens over time, so an early diagnosis is essential. The earlier the disease is diagnosed, the sooner you can start treatment that slows the progression of the disease.

An IPF doctor at an ILD Clinic can help ensure that you receive the treatment you need. Your doctor will give you a referral.

What are the possible treatments for IPF?

Currently, there is no cure for IPF. Once present in the lung, scarring cannot be reversed or removed. IPF treatments aim to slow the progression of the disease, manage physical symptoms and improve quality of life.

Treatment options for IPF include:

  • Lifestyle changes – it’s important to quit smoking, keep active, eat a healthy diet and have vaccinations for pneumonia and influenza to avoid illness
  • Medicines – to slow the scarring and preserve lung function
  • Pulmonary rehabilitation – includes exercise and education classes to help you conserve energy, exercise safely, manage breathlessness and maximise your quality of life
  • Oxygen therapy – to boost the oxygen level in your blood and help you breathe
  • Lung transplant – may be considered in some people
  • Clinical Trials – Your doctor may discuss the option of participating in a clinical trial.

What is the future plan if you have IPF?

For people living with IPF, the future depends on your age, overall health, lifestyle choices and the severity of the disease.

Some people with IPF get sick very quickly. Others may progress slowly.

Clinical trials looking at new IPF treatments offer a way for people to try new medicine that isn't yet available to everyone. Talk to your medical team about possible clinical trials.

St Vincent’s Hospital IPF Research

St Vincent’s Hospital engages in various local, national and international research, studying conditions such as IPF. You may have the opportunity to participate in one of the studies.

If you would like to be involved in IPF research at St Vincent’s Hospital, speak with your respiratory doctor to determine if there are any suitable studies.

For more information, email SVHS.lungresearch@svha.org.au