Idiopathic pulmonary fibrosis (IPF)

Treatment for IPF

Your specialist team will try to slow the scarring and treat your symptoms so you feel better and your quality of life improves.

You should have hospital appointments every three to six months and your care might include:

  • pulmonary rehabilitation  - a tailored exercise programme, which will help you cope with feeling short of breath
  • oxygen therapy – if the level of oxygen in your blood falls, you can have a portable oxygen cylinder or an oxygen concentrator at home, to make the air you breathe richer in oxygen
  • medication to help with symptoms
  • medication to slow the scarring in your lungs
  • help to stop smoking, if you smoke

For a very few people, having a lung transplant might be an option if the IPF progresses and isn’t controlled by treatment. Transplants are rare. Your specialist doctor should discuss lung transplantation with you within six months of being diagnosed, if it’s suitable for you. And, if you wish to explore the possibility, your doctor will contact the transplant centre.

Treating symptoms

For coughing, your doctor might treat problems that are making it worse, such as heartburn (acid reflux) or a stuffy nose. Make sure you tell your doctor if you have heartburn – there’s some evidence this may make your fibrosis worse as well as your cough.

To help you cope when you get out of breath, pulmonary rehabilitation is an important treatment and you’ll learn breathing techniques so that you feel more in control.

N-acetyl-cysteine or NAC helps break up mucus in the lungs, so your doctor may recommend it if you find it difficult to cough up phlegm or mucus. There’s anecdotal evidence that some people find it helps their cough, but others experience stomach discomfort, trapped wind or nausea.

Medication to slow scarring in your lung

There are currently two drugs which are licensed for use in IPF: pirfenidone and nintedanib. They both slow down the development of scar tissue in the lungs of people with IPF.

Several clinical trials are currently looking at possible new treatments, including combinations of existing treatments, so other options may be available in the coming years.

The National Institute for Health and Care Excellence (NICE), which advises the NHS on using new drug treatments, has only recommended the use of pirfenidone and nintedanib for people whose lung function is within a certain range. This means there’s a chance that your doctor may not be able to prescribe you either drug - they will explain why.

NICE has also recommended that if your IPF continues to get worse, these drug treatments should be stopped. Again, your consultant will discuss this with you.

Pirfenidone and nintedanib have not been directly compared in clinical trials. Your lung specialist will be able to discuss the pros and cons with you to help you decide which drug is best for you.

Pirfenidone - brand name Esbriet

This treatment comes in the form of capsules: the usual dosage is nine capsules each day (three taken with each meal).

Scientists don’t know exactly how pirfenidone works yet, but they think it slows down inflammation and the build-up of scar tissue in the lungs. In medical studies, it slowed down the loss of lung function in most people with IPF, decreased the rate at which their symptoms got worse and also improved life expectancy.

There are some common side effects. These are skin reactions to sunlight, feeling sick or nauseous, tiredness and indigestion. Talk to your doctor about possible side effects if you’re considering taking pirfenidone.

Nintedanib – brand name Ofev

Nintedanib is a new treatment which has also been shown in trials to slow the rate at which lungs become scarred in IPF. This drug is taken in the form of capsules, usually two a day.

Trials indicated nintedanib slows down the loss of lung function in people with IPF and may also reduce the rate of sudden flare-up of the symptoms.

Common side effects include diarrhoea and nausea. If you’re taking certain medications such as blood thinners, you may be advised not to take nintedanib.

There’s lots of research underway. For the most up-to-date information, call our helpline

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Last medically reviewed: September 2016. Due for review: September 2019

This information uses the best available medical evidence and was produced with the support of people living with lung conditions. Find out how we produce our information. If you’d like to see our references get in touch.