Idiopathic pulmonary fibrosis (IPF) can be hard to diagnose because its symptoms are similar to other lung conditions, such as chronic obstructive pulmonary disease (COPD).

A GP can refer you to hospital specialists for a number of tests to help rule out other conditions and confirm the diagnosis.

Medical history and examination

Your doctor will ask about your medical history and whether there are other factors that could be causing a problem with your lungs, such as whether you:

• smoke or have smoked in the past
• have been exposed to harmful substances at work, such as asbestos
• have other medical conditions

They may also:

• check the sound of your breathing through a stethoscope – a crackling sound can suggest lung scarring (fibrosis)
• look at your fingers to see if the ends are swollen (finger clubbing)
• ask you to walk around for a few minutes to see if you become breathless

Breathing and blood tests

Lung function tests (also called pulmonary function tests) assess how well your lungs work and can help indicate what the problem may be.

These tests measure:

• how quickly you can move air in and out of your lungs
• how much air your lungs can hold
• how well your lungs transfer oxygen into your blood and remove carbon dioxide from it (this can be checked using a blood test)

A commonly used lung function test is spirometry. During the test, you breathe into a mouthpiece that's attached to a monitor.

Chest X-ray and CT scan

A chest X-ray does not show the lungs in much detail, but can help doctors spot some more obvious problems that could be causing your symptoms, such as cancer or a build-up of fluid.

If IPF is suspected, the chest X-ray will be followed by a CT scan.

A CT scan is similar to an X-ray, but lots more images are taken and these are put together by a computer to create a more detailed image of your lungs.

This can help your doctor spot signs of scarring in your lungs.

Bronchoscopy

If doctors are still not sure what the problem is after these tests, they may suggest having a bronchoscopy.

This is a test where a narrow, flexible tube with a camera (bronchoscope) is passed down into your airways.

Your doctor will look for anything abnormal and may take small tissue samples for testing.

You'll usually be awake during a bronchoscopy and it may cause coughing.

Local anaesthetic will be used to numb your throat so it does not hurt, and you may also be given a sedative injection that'll make you feel sleepy during the procedure.

Lung biopsy

If other tests are not conclusive, a lung biopsy may need to be carried out.

This involves keyhole surgery to remove a small sample of lung tissue so it can be analysed for signs of scarring.

This is performed under a general anaesthetic, where you're asleep. 

Your surgeon makes several small cuts in your side and an endoscope, a thin tube with a camera and a light at the end, is inserted into the area between the lungs and the chest wall.

The surgeon can see the lung tissue through the endoscope and is able to obtain a small sample.

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 jab 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.

Some people also take a medicine called N-acetylcysteine, although its benefits are uncertain.

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

For more information, see the National Institute for Health and Care Excellence (NICE) guidelines on 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

For more information, see the NICE guidelines on nintedanib for idiopathic pulmonary fibrosis.

N-acetylcysteine

N-acetylcysteine is what's known as an antioxidant. It's available from many health shops and is usually taken as tablets.

There's some limited evidence to suggest it may reduce the amount of scar tissue in the lungs, although other studies have not shown any benefit.

If you're considering taking N-acetylcysteine, you should check with your specialist first as it may not be safe or suitable for everyone with IPF.

Oxygen support

IPF can cause levels of oxygen in your blood to 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.

Find out more about home oxygen treatment

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 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 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

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's 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 have 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.

Find out more about end of life care