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Pulmonary fibrosis

What is pulmonary fibrosis?

Pulmonary fibrosis is the end result of many different conditions that cause scar tissue to build up in your lungs and make breathing increasingly difficult. All types of pulmonary fibrosis are considered rare.

Pulmonary fibrosis is an interstitial lung disease (ILD). There are more than 200 different ILDs.

  • pulmonary: it affects your lungs
  • fibrosis: a build-up of scar tissue, which makes your lungs stiff
  • interstitial: affects your interstitium, the network of tissue that supports the air sacs in your lungs

What are interstitial lung diseases?

Interstitial lung diseases cause scarring in your lungs, inflammation in your lungs or a mix of both. Some mostly cause scarring. Some mostly cause inflammation. Often there is a combination of scarring and inflammation. It’s important to know which is the major cause of your symptoms to help decide the best treatment for you.

Causes and related conditions

Some types of pulmonary fibrosis have an identifiable cause. But for many types, a definite cause cannot be found, for example idiopathic pulmonary fibrosis.

Although we do not always know what causes pulmonary fibrosis, we do know it is not a form of cancer or cystic fibrosis, and it is not contagious. Cystic fibrosis is also not a type of ILD or pulmonary fibrosis.

This information covers the conditions seen most often:

Questions about diagnosis and treatment?

The treatment and outlook for different types of pulmonary fibrosis vary considerably, so if you’re not sure about your diagnosis, check with your doctor or nurse for the exact name of your lung condition.

Not sure what to ask?

If you are unsure what to ask your health care professional when you have, or could have, pulmonary fibrosis, try reading this helpful list. It suggests questions to ask for diagnosis, treatments and much more.

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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.