Who can get IPF?
What are the symptoms?
How is it diagnosed?
Treatment
Idiopathic pulmonary fibrosis (IPF) used to be known fibrosing alveolitis and also cryptogenic fibrosing alveolitis. It is caused by repeated harm to small areas of your lung. This results in scarring.
Although the harm at any one time is to a small part of your lung, the effects build up until they affect enough of your lung to cause symptoms. Along with the injury and the scarring, there is also inflammation. This results in further injury, and makes your lung function worse.
Scars serve a good purpose in the skin - they heal injured areas. But in the lung, scar tissue stops the lung doing its job - taking oxygen from the air and passing it into the blood. So you may have breathing difficulties or get breathless from simple activities such as walking and talking.
It is important to note that IPF is not cancer. It is not infectious either.
IPF cases seem to be rising, but it's not clear why. It affects men and women of any age, but it's most common among people in their 50s.
We don't know what causes IPF. But we do know that:
- it is not an infection
- it can not be caught from other people
- it is not a form of cancer
- it is not a form of cystic fibrosis
A number of factors appear to 'trigger' the condition, but they do not cause it. Some drugs can also scar the lung, but this is not IPF. Occasionally, the disease happens to several people in the same family. The genetic reasons for this remain unknown, however, as it doesn't happen in a predictable way.
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The most common symptom is breathlessness, particularly during activities like walking up hills or stairs. IPF is most common among people in their 50's, and some people think that getting breathless is just part of getting older. But you shouldn't think that. Breathlessness has to be investigated. Otherwise, if you do have a condition it will often get worse. This can lead to permanent breathlessness which will go on getting worse.
Less common symptoms include a dry cough, and many people may notice a change in the shape of their finger and toe nails. Occasionally IPF is only noticed when people have a chest X-ray for some unrelated reason.
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Although many cases are not diagnosed, IPF is still one of the less common lung diseases. It only affects one in every 5,000 - 10,000 people. This means that your GP will see very few people with it, or possibly none at all. So you will be referred to a specialist.
Tests usually include:
- a scan of your lungs called a 'high resolution CT scan'
- breathing tests to see how well your lungs are working
- a blood test
- it may be necessary to obtain samples of lung tissue. This is usually done by bronchoscopy, which involves passing a small flexible telescope (called a bronchoscope) down your breathing tubes - you will have a mild sedative while this is done.
For many people these tests are enough for planning treatment, but in some people doctors need a larger sample of lung tissue. This will be taken by a surgeon, and you will have a general anaesthetic.
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IPF usually needs treatment even if your symptoms appear mild. This is because by the time you have symptoms, your lungs are already badly damaged. It's very important that the disease is caught as early as possible, so that treatment can start. Treatment is more effective in IPF when it is given before extensive lung scarring has happened.
The type of treatment you will have depends on the results of your tests. The most common form of treatment is low-dose steroids and drugs which suppress the body's defence mechanisms. Doctors believe our defences contribute to the disease.
This treatment can also make you more vulnerable to infection. People who are taking these drugs will need to have their blood checked on a regular basis to make sure that they are not receiving too much treatment. When the drugs reach their most effective level, the aim is to keep that effectiveness while reducing the treatment.
A few people may experience side effects; so your doctor will weigh up the potential benefit of treatment and the risk of side effects.
In IPF, you will usually need treatment for all of your life. This is because for most people the treatment suppresses the condition rather than cures it. The long-term aim is to keep the disease suppressed using the smallest possible dosage of treatment. Very occasionally, drugs may be stopped completely - but this is exceptional.
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Last medically reviewed: February 2006
