There is currently no cure for idiopathic pulmonary fibrosis. The main aim of treatment is to improve the symptoms of the disease as much as possible and slow down its progression.
Medication can help to reduce symptoms in some people, but does not work for everyone. Increasing evidence suggest that medications such as steroids and immunosuppresants do more harm than good. Some people, particularly elderly people whose symptoms are mild or not getting much worse, may be advised not to take any medication. This is because possible side effects could be worse than the progression of the disease.
Steroid medication is thought to improve symptoms in about one person in four with idiopathic pulmonary fibrosis. A low dose of a corticosteroid called prednisone may be prescribed. If there is no improvement after a few months, the treatment may be stopped.
Taking steroid medication, especially for a prolonged period of time, can cause side effects including:
- weight gain
- osteoporosis (brittle bones, which especially affects women after the menopause)
- cataracts (cloudy patches in the lens in the eye)
- glaucoma (an eye disease that impairs vision)
- stomach problems
It can also trigger diabetes and raised blood pressure. For more information, see the Health A-Z page on Side effects of corticosteroids.
Your doctor or specialist should always weigh up the potential benefits of steroid treatment against the risk of side effects.
Immunosuppressants are drugs that suppress the body's immune system (thought to be partly responsible for idiopathic pulmonary fibrosis), helping to stop inflammation and fibrosis. You may be prescribed the immunosuppressant drugs cyclophosphamide or azathioprine.
Taking immunosuppressant drugs can increase your likelihood of getting other infections and can cause problems with your blood, such as anaemia.
Again, your doctor or specialist should always weigh up the potential benefits of immunosuppressant treatment against the risk of side effects.
A combination of steroids and an immunosuppressant may be used in some cases as there is thought to be more chance of improvement when these drugs are used together than when either medication is used on its own. Combination therapy also allows for the dose of steroids to be lower, meaning there is less chance of severe side effects.
When idiopathic pulmonary fibrosis is severe, the 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 you are connected.
You can also use an oxygen inhaler for when you are out and about.
Pulmonary rehabilitation is a multidisciplinary treatment (involving many different types of healthcare professionals) used for many long-term lung conditions. Courses aim to help a patient 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 above video in which a respiratory nurse specialist and patients explain pulmonary rehabilitation.
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. For more information on the procedure, see the Health A-Z topic on lung transplant.
As well as the medical treatments above, there are measures you can take to stay as healthy as possible:
- stop smoking
- exercise regularly and stay as fit as you can
- eat a good, balanced diet
- take the flu jab and the pneumococcal vaccination (influenza and pneumococcal bacterial infections can become severe if you have a lung disease)
- try to keep away from people with chest infections and colds