Treating idiopathic pulmonary fibrosis 

There is currently no cure for idiopathic pulmonary fibrosis (IPF). The main aim of treatment is to relieve symptoms of the disease as much as possible and slow down its progression.

The best treatment will depend on how severe your illness is and your own preferences.

You may also need treatment for any other conditions you have. In some cases, you may need to stop certain treatments if they are not working or if you experience severe side effects.

Self care

As well as medical treatments, there are measures you can take to stay as healthy as possible:

Treating breathlessness

If you get out of breath regularly, your doctor may recommend you are assessed for either:

  • oxygen support
  • pulmonary rehabilitation

Oxygen support

When IPF is severe, levels of oxygen in your blood fall and you feel more breathless. In this case your specialist may prescribe you oxygen, which can help with your breathing and enable you to be more active.

You will need to take oxygen through nasal tubes or a mask from a portable machine called an oxygen concentrator. This device provides air with a much higher oxygen level than the air you breathe in naturally. The tubes from the machine are long, so you will be able to move around your home while connected.

Read more information about home oxygen treatment.

You can also use an oxygen inhaler when you are out and about.

Pulmonary rehabilitation

Pulmonary rehabilitation is used for many long-term lung conditions and aims to help patients come to terms with their condition, learn the best ways to deal with it and improve their ability to function on a daily basis without extreme breathlessness.

Courses in pulmonary rehabilitation are usually held locally and may include:

  • education about pulmonary fibrosis
  • physical exercise
  • breathing exercises
  • advice on nutrition
  • psychological support
  • a social support network

For more information, watch the video on this page in which a respiratory nurse specialist and patients explain pulmonary rehabilitation.

Medication

Medication can help reduce symptoms of IPF in some people, but they're not suitable for everyone.

Some people, particularly elderly people whose symptoms are mild or not getting worse, may be advised not to take any medication. This is because possible side effects could be worse than the progression of the disease.

The medications your doctor may recommend to treat IPF include:

  • pirfenidone
  • N-acetylcysteine
  • nintedinib

Pirfenidone

Pirfenidone helps to slow the development of scarring in the lungs by suppressing the immune system.

The National Institute for Health and Care Excellence (NICE) recommends that pirfenidone may be used for treating adults with IPF who have a lung capacity of 50-80% of the expected value.

If your disease gets worse and your lung capacity falls by 10% or more within a year, then pirfenidone should be stopped.

Side effects of pirfenidone may include:

  • nausea
  • vomiting
  • diarrhoea
  • abdominal pain
  • tiredness (fatigue)
  • photosensitivity rash (on exposure to sunlight)

Read the NICE guidelines on pirfenidone for idiopathic pulmonary fibrosis (PDF, 161kb).

N-acetylcysteine

N-acetylcysteine is what is known as an antioxidant. There is currently some limited evidence to suggest this medication can reduce the amount of scar tissue created in the lungs.

N-acetylcysteine is available from many health shops on an over-the-counter basis and is taken as tablets, sometimes dissolved in water.

Check with the doctor in charge of your care before taking this medication as it may not be safe or suitable for everyone with IPF.

Nintedinib

Nintedinib is a newer medication, known as an anti-fibrotic kinase inhibitor, which has shown promising results in recent trials.

Although it is currently unlicensed, certain people with IPF may be offered nintedinib if their doctor thinks they could benefit from using it.

Lung transplant

If your condition continues to get worse despite other forms of treatment, your consultant may recommend a lung transplant. The decision to have a transplant will be based on:

  • how bad your condition is
  • how quickly your condition is getting worse
  • your age and general health
  • how much your condition is likely to improve after a transplant
  • whether a donor lung is available

Few people with idiopathic pulmonary fibrosis are suitable candidates for a lung transplant.

Pulmonary rehabilitation

A respiratory nurse specialist and patients explain pulmonary rehabilitation and how exercise can improve the symptoms of COPD.

Media last reviewed: 19/07/2015

Next review due: 19/07/2017

End of life care

Being told you have a condition likely to prove fatal can be a terrible shock. Many people reported feeling a wide range of emotions from numbness and confusion to fear.

Most people come to terms with the diagnosis over time and begin to think about issues related to end of life care, such as:

  • their financial affairs
  • where they would like to be treated in their final months
  • types of treatment they would be willing to consider

For more information see the NHS Choices End of life care guide.

Page last reviewed: 04/07/2014

Next review due: 04/07/2016