Idiopathic pulmonary fibrosis (IPF)

What is IPF?

Idiopathic pulmonary fibrosis (IPF) is a lung condition that scars your lungs and reduces the efficiency of your breathing. It’s the most common type of pulmonary fibrosis.

What does IPF stand for?

  • idiopathic because no one knows what causes it
  • pulmonary because it affects your lungs
  • fibrosis because that’s what the scar tissue in your lungs is called

IPF is a progressive condition and usually gets worse over time. At present, the scar tissue cannot be repaired by the body or any drugs, so there is no cure yet for IPF.

Current treatment focuses on trying to slow the rate scar tissue is formed and controlling symptoms. In some people, symptoms get worse gradually over several years. For others, the symptoms get worse more quickly.

It’s difficult to predict how rapidly IPF will progress in each person affected. Sometimes when the condition has been stable, people can get sudden flare-ups of symptoms, called exacerbations.

There are no defined stages of IPF though some people may talk about mild, moderate or severe disease. Everyone is different - talk to your specialist doctor about your individual situation.

How does IPF affect breathing?

Scar tissue causes the lungs to become stiffer and to lose their elasticity. This stops them functioning properly: they’re less able to inflate and transfer oxygen from the air you breathe into your body.

Each time you breathe in, you draw in air through your nose and mouth, down through your throat and into your windpipe (trachea). Your windpipe splits into two main tubes, the right and left bronchi, which supply your lungs. The main bronchi divide into gradually smaller airways called bronchioles. Bronchioles have many small air sacs (alveoli) at their ends. Inside the air sacs, oxygen moves across paper-thin walls to tiny blood vessels and into your blood. The air sacs also exchange waste gas, carbon dioxide, from your blood ready for you to breathe it out.

Diagram of the lungs, airways, bronchioles, and air sacs

If you have IPF, scarring affects the air sacs, eventually limiting the amount of oxygen that gets into the blood. With less oxygen in the blood, you can get breathless from everyday activities like walking. 

Air sacs of the lungs in pulmonary fibrosis

 

Next: What causes IPF >

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Last medically reviewed: August 2019. Due for review: August 2022

This information uses the best available medical evidence and was produced with the support of people living with lung conditions. Find out how we produce our information. If you’d like to see our references get in touch.