Idiopathic pulmonary fibrosis

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 known. 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.

Other lung diseases

As well as idiopathic pulmonary fibrosis, there are other forms of fibrotic lung disease. These include Non Specific Interstitial Pneumonia (NSIP), Hypersensitivity Pneumonitis, Connective Tissue Disease related Interstitial Lung Disease, Vasculitis, Asbestosis and Sarcoidosis.

It’s possible to have other chronic lung diseases as well as fibrotic lung disease. This includes:

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

End of life care

If you’re told there is nothing more that can be done to treat your IPF or you decide you don’t want treatment, your GP or care team will give you support and treatment to relieve your symptoms. This is called palliative care.

Read further information about palliative care.

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Your GP may ask if you smoke, or if you used to, whether you have ever been exposed to harmful substances such as asbestos at work, and whether you have any other medical conditions.

They may also listen to your breathing with a stethoscope, since a crackling sound can suggest scarring in the lungs (fibrosis). They may look at your fingers to see if the tips are swollen, a sign known as finger clubbing, and ask you to walk around for a few minutes to see if you become breathless. A chest X-ray is often arranged as well.

Tests

If your GP thinks you could have a lung condition like IPF, they can refer you to a hospital specialist for further tests. These usually include lung function tests to check how well your lungs are working, blood tests, and a computerised tomography (CT) scan to look for anything that could be causing your symptoms, such as cancer, a build up of fluid, or scarring.

Usually, IPF can be diagnosed without any further tests. Sometimes, though, your specialist may consider other tests such as a bronchoscopy, in which 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. They might also suggest a lung biopsy, where a small piece of lung tissue is removed during keyhole surgery so it can be analysed, or an echocardiogram.

There's no cure yet for IPF, but several treatments can ease symptoms and slow its progress.

These include:

• lifestyle changes, such as quitting smoking, eating well and staying active
• medicines like pirfenidone and nintedanib, which slow the rate of scarring. Your specialist team will often track your breathing tests and scans over time to see how the disease is progressing before starting these
• breathing oxygen through a mask. If your oxygen levels are low at rest, your team may suggest wearing oxygen all the time; if they only drop when you're moving about, you may only need it when you're out and active
• pulmonary rehabilitation, which uses exercises and guidance to help you breathe more comfortably
• a lung transplant, suitable only in some cases, since donor lungs are scarce and patients need to be otherwise quite fit to benefit