Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a condition where the lungs become scarred, and breathing gets harder as a result.

Doctors don't know what causes it. It usually affects people around 70 to 75 years old, and it's rare in people under 50. The older you are, the more likely you are to develop IPF.

Antifibrotic treatment can help slow the condition down. But there's no treatment at present that can stop or reverse the scarring of the lungs.

Idiopathic pulmonary fibrosis (IPF) usually starts slowly, and the symptoms get worse bit by bit over time.

The 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. They put it down to getting older or being out of shape. But in time, even light activity like getting dressed can leave you short of breath.

Idiopathic pulmonary fibrosis (IPF) puts a strain on your lungs and heart, and that strain raises the risk of other health problems. These include:

• chest infections and airway infections, for example bronchitis, pneumonia and influenza (flu)
• pulmonary hypertension
• right sided heart failure

See a doctor if you have:

• struggled with your breathing for a while
• had a cough for more than 3 weeks

Your doctor may ask if you:

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

They may also:

• listen to your breathing through a stethoscope (a crackling sound can suggest lung scarring, which doctors call fibrosis)
• look at your fingers to see if the ends are swollen (this is called finger clubbing)
• ask you to walk around for a few minutes to see if you become breathless
• arrange a chest X-ray

If your doctor thinks you could have a lung condition like idiopathic pulmonary fibrosis (IPF), they can refer you to a hospital specialist for tests such as:

• lung (pulmonary function) tests to check how well your lungs work
• blood tests
• a computerised tomography (CT) scan to see if there's anything causing your symptoms, like cancer, a build up of fluid or scarring

Usually, IPF is diagnosed without the need for additional tests. Sometimes your specialist may consider other tests such as:

• bronchoscopy (where a narrow, flexible tube with a camera is passed down into your airways) to look for anything abnormal and take a tissue sample for testing
• a lung biopsy (where a small piece of lung tissue is removed during keyhole surgery so it can be analysed)
• an echocardiogram

There's no cure for idiopathic pulmonary fibrosis (IPF) at present, but several treatments can ease the symptoms and slow down how fast it progresses.

Treatments include:

• lifestyle changes, such as stopping smoking, eating healthily and exercising regularly
• medicine such as pirfenidone and nintedanib, to reduce the rate at which scarring worsens. Before starting treatment, your specialist team will often monitor you, your breathing tests and your scan over time to see whether your condition is progressing
• breathing oxygen through a mask. If your oxygen levels are low when you're resting, your specialist team may recommend wearing oxygen all the time. If they are low only when you move around, you may need it only when you're out and about
• pulmonary rehabilitation, which means exercises and advice to help you breathe more easily
• a lung transplant, which is suitable in a few cases. Donor lungs are rare, and patients have to be otherwise very fit to benefit from a transplant

In idiopathic pulmonary fibrosis (IPF), the tiny air sacs in the lungs (alveoli) become damaged and more and more scarred. The lungs turn stiff, and it gets hard for oxygen to pass into the blood.

Nobody knows why this happens. That's what "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
• gastro-oesophageal reflux disease (GORD)
• smoking

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

Idiopathic pulmonary fibrosis isn't the only kind of fibrotic lung disease. There are others, including Non Specific Interstitial Pneumonia (NSIP), Hypersensitivity Pneumonitis, Connective Tissue Disease related Interstitial Lung Disease, Vasculitis, Asbestosis and Sarcoidosis.

You can also have other chronic lung diseases alongside a fibrotic lung disease. These include:

• bronchiectasis
• asthma
• chronic obstructive pulmonary disease (COPD), which is known as Combined Pulmonary Fibrosis and Emphysema (CPFE)

Sometimes doctors tell you there is nothing more they can do to treat your idiopathic pulmonary fibrosis (IPF). Or you may decide you don't want treatment. Either way, your doctor or care team will still support you and treat your symptoms to give you relief. This is called palliative care.