What is idiopathic pulmonary fibrosis? | Action for Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive lung disease. Studies suggest there are about 30,000 people living with IPF in the UK.

IPF is the commonest form of pulmonary fibrosis and it is estimated that there are 6,000 new cases of this condition each year in the UK. Studies suggest ere are about 30,000 people living with IPF in the UK. IPF usually develops in people aged 70 and older. It can occur in younger individuals particularly if there is a family history of idiopathic pulmonary fibrosis. It is more common in men.

Causes of idiopathic pulmonary fibrosis

In IPF, scar tissue or fibrosis builds up around the air sacs(alveoli) in the lungs and reduces their ability to transfer oxygen that is breathed in to the blood.

We do not know what causes IPF. Research suggests that fibrosis occurs in response to damage of the cells that line the air sacs (alveoli). It is likely due to a combination of genetic and environmental factors, such as smoking, dusts from metal, wood and agricultural work.

IPF is the commonest form of pulmonary fibrosis and it is estimated that there are 6,000 new cases of this condition each year in the UK. It usually develops in people aged 70 and older, and is more common in men. It can occur in younger individuals particularly if there is a family history of idiopathic pulmonary fibrosis.

IPF may run in families: it has been estimated that 5-20% of patients have a relative with the disease. This is termed familial pulmonary fibrosis.

Symptoms of idiopathic pulmonary fibrosis

People with idiopathic pulmonary fibrosis are usually breathless and may have a cough. They may also describe feeling tired or fatigue and weight loss. Pulmonary hypertension (high blood pressure in the arteries of the lungs) can occur as a consequence of low blood oxygen levels associated with IPF.

Find more about pulmonary fibrosis and its symptoms.

Treatments for idiopathic pulmonary fibrosis

Anti-fibrotic drugs are often prescribed for IPF to slow down the scarring process in the lungs and improve survival.

There are a number of treatments to help with symptom control. These include:

breathlessness management and pulmonary rehabilitation
oxygen therapy – if blood oxygen levels fall with exercise or when resting
cough suppressants to aid sputumclearance
management of anxiety and panic attacks

Your doctor may suggest assessment by palliative and supportive care specialists for helping with symptom control and psychological support.

For people with chronic lung disease, you may be advised to have a pneumonia vaccine and the seasonal flu vaccine.

If you smoke, there is help to stop smoking

For some people, referral for lung transplantation may be discussed.

There are a number of novel therapies being evaluated in clinical trials for IPF. Please discuss with your doctor if you are interested in finding out about clinical trials.

Ongoing treatment

Your medical team will monitor you regularly to understand how your IPF is progressing, and whether you require different treatment.

Outlook for idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis is a progressive and ultimately a terminal disease. Most patients may live three to five years after diagnosis. However, anti-fibrotic therapies may improve life expectancy.

IPF usually progresses steadily. But sometimes it can progress very rapidly, which is called an acute exacerbation. This can occur spontaneously or be triggered by a respiratory tract infection. If this happens, you must contact your ILD specialist nurse or GP urgently.

Other forms of pulmonary fibrosis

Get support

Join a support group in your area

It’s important to remember that you are not alone. There are now over 75 pulmonary fibrosis support groups around the country.

Support groups meet informally to share experiences, provide expert information and raise funds and awareness.

Call our support line

APF runs an email and telephone support line offering information and advice about living with PF.

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Watch videos of how other people cope day to day with PF.

Information provided by Action for Pulmonary Fibrosis (APF) is not a substitute for professional medical advice. It’s intended as general information only. APF is not responsible for any errors or omissions or for any loss or damage suffered by users resulting from the information published on actionpf.org.