Health A to Z
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 70-75 years of age 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.
This page covers:
The symptoms of IPF tend to develop gradually and get slowly worse over time.
Symptoms can include:
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.
See your GP if you've struggled with your breathing for a while or have had a cough for more than three weeks.
These symptoms aren't normal and shouldn't 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:
Read more about the tests for 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 isn't clear. "Idiopathic" means the cause is unknown.
IPF has been linked to:
But it's not known whether some of these factors directly cause IPF.
There's currently no cure for IPF, but there are several treatments that can help relieve the symptoms and slow down its progression.
Read more about how idiopathic pulmonary fibrosis is treated.
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.
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.
Before the availability of specific treatments like pirfenidone and nintedanib about half of people with IPF lived at least three years from their diagnosis. Around 1 in 5 survived for more than five years.
It's hoped these figures will be improved by the availability of new treatments to slow the disease's progression.
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).
Your GP can refer you to hospital specialists for a number of tests to help rule out other conditions and confirm the diagnosis.
The checks and tests you may have include:
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:
They may also:
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:
A commonly used lung function test is spirometry. During the test, you breathe into a mouthpiece that's attached to a monitor.
A chest X-ray doesn't show the lungs in much detail, but it 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 computerised tomography (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.
If doctors still aren't sure what the problem is after the tests described above, 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 can then look for anything abnormal and can take small tissue samples for testing.
You'll usually be awake for a bronchoscopy and it may cause coughing. Local anaesthetic will be used to numb your throat so it doesn't hurt and you may also be given a sedative injection which will make you feel sleepy during the procedure.
If other tests aren't 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.
The procedure is performed under a general anaesthetic (where you're asleep). Your surgeon makes several small incisions (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 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.
The main treatments are:
As the condition becomes more advanced, end of life (palliative) care will be offered.
There are several things you can do to stay as healthy as possible if you have IPF.
There are two medications that can help slow down the progression of IPF in some people: pirfenidone and nintedanib.
Some people also take a medication called N-acetylcysteine, although its benefits are uncertain.
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 three times a day.
It's recommended if breathing tests have shown your lung capacity is 50-80% 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 medication will usually be stopped.
Side effects of pirfenidone can include:
For more information see, the National Institute for Health and Care Excellence (NICE) guidelines on pirfenidone for idiopathic pulmonary fibrosis.
Nintedanib is a newer medication 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 50-80% 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:
For more information, see the NICE guidelines on nintedanib for idiopathic pulmonary fibrosis.
N-acetylcysteine is what's known as an antioxidant. It's available from many health shops and is usually taken as tablets.
There is 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.
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.
Read more about home oxygen treatment.
Portable oxygen devices that you can use while you're out and about are also available.
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:
For more information, watch this video about pulmonary rehabilitation.
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:
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.
If you're told there is nothing more that can be done to treat your IPF or you decide to decline treatment, your GP or care team will provide you with support and treatment to relieve your symptoms.
This is called palliative care.
You can choose to receive palliative care:
Your doctor or care team should work with you to establish a clear plan based on your wishes.
For more information see the NHS Choices End of life care guide.