Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a long-term condition that affects the lungs. In IPF, the lungs become thickened, scarred and stiff causing breathing problems. 

• Idiopathic (means a condition where other known causes have been excluded).
• Pulmonary (relating to the lungs or respiratory system).
• Fibrosis (thickening and scarring of tissue).
  
  

Image credit: NIH via Wikimedia Commons(external link)

The diagnosis can only be made after a full assessment and a number of investigations have been completed as requested by a respiratory specialist. Read more below and in the IPF diagnosis section. 

As the scarring is progressive and irreversible, most people with IPF get worse over time. Each person experiences IPF differently. Some people can remain stable for many years, others get worse quickly. Medicines such as pirfenidone and nintedanib help to slow the rate of disease progression.

Things to know about IPF

• There’s no known cause for IPF. It's not an infection, a form of cancer or anything to do with COPD, cystic fibrosis or bronchiectasis.
• Signs of lung scarring on a chest X-ray or a CT scan are not enough to make a diagnosis of IPF. A full, comprehensive assessment is needed.
• Several lung conditions (eg, sarcoidosis, rheumatoid arthritis and scleroderma) may result in progressive lung scarring (fibrosis). But these conditions are treated differently.
• Several occupational lung diseases (eg, asbestosis or silicosis) and allergic lung conditions (eg, bird-fancier's lung) can also result in progressive pulmonary fibrosis. They too are treated quite differently and it's very important to have these ruled out before a formal diagnosis of IPF is made.
• IPF usually affects people over 60 years of age, and is rare in people under 50. Men are slightly more often affected than women. 
• IPF is a serious and potentially fatal condition. 

The symptoms of IPF tend to develop gradually and slowly get worse over time.

Symptoms can include:

• shortness of breath – especially with activity such as walking up hills or stairs 
• a persistent dry or hacking cough
• tiredness, fatigue and weakness
• loss of appetite and weight loss
• wide, rounded and swollen fingertips (clubbed fingers), but there are a number of other medical conditions associated with finger clubbing.
  
  

Image credit: Canva

Many people ignore their breathlessness at first and blame it on getting old or being out of shape. But eventually even light activity, such as getting dressed, can cause shortness of breath. Despite this, exercise is encouraged in order to break the cycle of feeling breathless, avoiding activities and then becoming even more breathless. 

Each person experiences IPF differently. How quickly the symptoms develop and how severe they get can vary greatly from person to person. You may remain stable for many years or become ill very quickly with severe disease. Alternatively, you may have moderate symptoms that get worse slowly, over months or years.

As the scarring develops and can’t be reversed, most people with IPF get worse over time. A few people experience acute flare ups or exacerbations. These may be fatal.

IPF may be difficult to diagnose as the symptoms are similar to other lung diseases. For this reason a formal diagnosis of IPF should only be made by an expert panel of respiratory specialists at a larger hospital in Aotearoa New Zealand.

If your healthcare provider thinks you could have IPF, you should be referred to a respiratory specialist for tests. These may include:

• breathing (lung function) tests to assess your breathing and how well your lungs are working
• blood tests to rule out other conditions associated with pulmonary fibrosis
• a chest CT providing a 3D radiological assessment of your lungs.
• a lung biopsy, where a small piece of lung tissue is removed for testing – it may be done with a bronchoscopy or by video-assisted thoracoscopic lung surgery VATS (keyhole surgery).

The lung scarring and thickening that occurs in pulmonary fibrosis can't be repaired. There's currently no cure for IPF, but there are several treatments that can help improve your symptoms, and slow down the progression of the disease. Stopping smoking is essential. It's also important to avoid any lung irritants, such as dust or fumes, as they can make your symptoms worse.  

Medicines

Medicines such as pirfenidone and nintedanib help to reduce the rate at which scarring worsens. These medicines are called anti-fibrotic medicines. Specific criteria apply and they can only be prescribed by lung specialists. Regular follow-up assessments are required in order to continue these treatments.

Oxygen

If your oxygen levels are low, breathing oxygen using nasal cannula can make breathing and exercise easier. It can prevent strain on your heart and reduce complications from low oxygen levels. There are strict criteria for starting long term oxygen therapy. These criteria are set down by the Ministry of Health and include:

• a no smoking policy (it can be very dangerous because of the fire risk), and
• clear cut-offs for oxygen levels measured by an arterial blood gas test (ABG).

Read more about home oxygen therapy. 

Pulmonary rehabilitation

Pulmonary rehabilitation is a program of exercise training, education and support, that teaches you the skills you need to manage your breathlessness, to stay well and out of hospital. The programme is provided by specially trained health professionals. Daily exercise for at least 20 to 30 minutes is usually encouraged. Read more about pulmonary rehabilitation. 

Lung transplantation

A lung transplant may be needed if you have very severe disease or other treatment approaches have been tried without success. In Aotearoa New Zealand this can only be performed at Auckland Hospital and strict criteria apply. All hospital respiratory specialists can make referrals for lung transplantation.