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Idiopathic pulmonary fibrosis (IPF)

What is IPF?

Idiopathic pulmonary fibrosis (IPF) is a lung condition that scars your lungs and makes it difficult for you to breathe. It’s the most common type of pulmonary fibrosis. 

What does IPF stand for? 

• idiopathic = there is no known cause 

• pulmonary = it affects your lungs 

• fibrosis = the name of the scar tissue in your lungs

IPF is a progressive condition. This means it gets gradually worse over time. The scar tissue in the lungs cannot be repaired by the body or any drugs, and there is currently no treatment that stops or reverses the scarring.  

Current treatment focuses on slowing the rate of scarring in the lungs and controlling symptoms. Some people respond well to treatment and find their symptoms remain the same for many years. For others, the symptoms get worse more quickly.     

It’s difficult to predict how quickly IPF will develop because it varies a lot from person to person.  There are no clear stages of IPF, though some people may talk about mild, moderate, or severe disease. Everyone is different - talk to your specialist doctor about your own situation.

How does IPF affect breathing? 

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

Each time you breathe in, you take 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 with air. 

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. 

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. 

Read next: what causes IPF? 

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