Idiopathic pulmonary fibrosis (IPF) is a condition in which the lungs become scarred and breathing becomes increasingly difficult.

It's not clear what causes it, but it usually affects people around 70 to 75 years old and is rare in people under 50.

Several treatments can help reduce the rate at which IPF gets worse, but there's currently no treatment that can stop or reverse the scarring of the lungs.

Symptoms of idiopathic pulmonary fibrosis

The symptoms of IPF tend to develop gradually and get slowly worse over time.

Symptoms can include:

• shortness of breath
• a persistent dry cough
• tiredness
• loss of appetite and weight loss
• rounded and swollen fingertips (clubbed fingers)

Many people ignore their breathlessness at first and blame it on getting old or being out of shape.

But eventually even light activity such as getting dressed can cause shortness of breath.

When to get medical advice

See a GP if you've struggled with your breathing for a while or have had a cough for more than 3 weeks.

These symptoms are not normal and should not be ignored.

If your GP thinks you could have a lung condition such as IPF, they can refer you to a hospital specialist for tests such as:

• breathing (lung function) tests
• blood tests
• a chest X-ray and CT scan
• a lung biopsy, where a small piece of lung tissue is removed during keyhole surgery so it can be analysed

Causes of idiopathic pulmonary fibrosis

In people with IPF, the tiny air sacs in the lungs (alveoli) become damaged and increasingly scarred.

This causes the lungs to become stiff and means it's difficult for oxygen to get into the blood.

The reason this happens is not clear. Idiopathic means the cause is unknown.

IPF has been linked to:

• exposure to certain types of dust, such as metal or wood dust
• viral infections
• a family history of IPF – around 1 in 20 people with IPF has another family member with the condition
• acid reflux
• smoking

But it's not known whether some of these factors directly cause IPF.

Treatments for idiopathic pulmonary fibrosis

There's currently no cure for IPF, but there are several treatments that can help relieve the symptoms and slow down its progression.

Treatments include:

• self-care measures – such as stopping smoking, eating healthily and exercising regularly
• medicines to reduce the rate at which scarring worsens, such as pirfenidone and nintedanib
• breathing oxygen through a mask – you can do this while you're at home or while you're out and about
• exercises and advice to help you breathe more easily (pulmonary rehabilitation)
• a lung transplant – this is suitable in a few cases, although donor lungs are rare

Further information

UK Charities Action for Pulmonary Fibrosis and the Pulmonary Fibrosis Trust provide information and support for people affected by IPF as well as news items about ongoing research into the condition.

Outlook for idiopathic pulmonary fibrosis

IPF gets worse over time, although the speed at which this happens is highly variable.

Some people respond well to treatment and remain relatively free of symptoms for many years, while others may get rapidly worse or find the breathlessness debilitating.

Other problems can also sometimes develop, including chest infections, pulmonary hypertension and heart failure.

It's very difficult to predict how long someone with IPF will survive at the time of diagnosis.

Regular monitoring over time can indicate whether it's getting worse quickly or slowly.

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

As the condition becomes more advanced, end of life (palliative) care will be offered.

Self-care

There are several things you can do to stay as healthy as possible if you have IPF.

These include:

• stopping smoking if you smoke
• exercising regularly and staying as fit as you can
• eating a healthy, balanced diet
• making sure you get the annual flu vaccine and one-off pneumococcal vaccination – these infections can be more serious if you have a lung condition
• try to keep away from people with chest infections and colds whenever possible

Medicines

There are 2 medicines that can help slow down the progression of IPF in some people: pirfenidone and nintedanib.

Pirfenidone

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

It's normally taken as capsules 3 times a day.

It's recommended if breathing tests have shown your lung capacity is 50% to 80% of what would normally be expected.

If your condition gets worse despite taking pirfenidone and your lung capacity falls by 10% or more within a year, the medicine will usually be stopped.

Side effects of pirfenidone can include:

• feeling sick
• tiredness
• diarrhoea
• indigestion
• a rash caused by exposure to sunlight

There have been reports of pirfenidone causing serious liver damage. While this risk is thought to be low, serious liver damage can be fatal.

If you’re prescribed pirfenidone, you’ll have blood tests to check your liver function before starting treatment and at regular intervals during treatment.

Symptoms of liver damage include:

• yellowing of the eyes or skin
• loss of appetite
• itchy skin
• your pee turns a darker colour
• feeling tired
• pain in the upper right side of your stomach

If you have any of these symptoms, then stop taking your medicine and talk to your doctor immediately. If this is not possible call 111 for advice.

See the National Institute for Health and Care Excellence (NICE) guidelines about pirfenidone for idiopathic pulmonary fibrosis

Nintedanib

Nintedanib is a newer medicine that can also help slow down scarring of the lungs in some people with IPF. 

It's normally taken as capsules twice a day.

Like pirfenidone, it may be used if you have a lung capacity 50%  to 80% of what would normally be expected and should be stopped if your lung capacity falls by 10% or more in a year while taking it.

Side effects of nintedanib can include:

• diarrhoea
• feeling and being sick
• tummy (abdominal) pain
• loss of appetite and weight loss

See the NICE guidelines about nintedanib for idiopathic pulmonary fibrosis

Oxygen support

IPF can cause levels of oxygen in your blood fall, which can make you feel more breathless.

If this happens, oxygen treatment can help with your breathing and allow you to be more active.

Oxygen is taken through nasal tubes or a mask attached to a small machine. This device provides a much higher level of oxygen than the air.

The tubes from the machine are long, so you'll be able to move around your home while connected.

Portable oxygen devices that you can use while you're out and about are also available.

Pulmonary rehabilitation

Pulmonary rehabilitation is used for many long-term lung conditions.

It 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 severe breathlessness.

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

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

Find out more about pulmonary rehabilitation on the Asthma and Lung UK website

Lung transplant

If your condition continues to get worse despite treatment, your specialist 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

A lung transplant can significantly improve life expectancy in people with IPF, although it's a major procedure that puts a great strain on the body.

Few people with idiopathic pulmonary fibrosis are suitable candidates for a transplant and donor lungs are scarce.

Palliative care

If you're told there is nothing more that can be done to treat you, or you decide not to have treatment, your GP or care team will give you support and treatment to relieve your symptoms.

This is called palliative care.

You can choose to receive palliative care:

• at home
• in a care home
• in hospital
• in a hospice

Your doctor or care team should work with you to establish a clear plan based on your wishes.