Pulmonary fibrosis

Pulmonary fibrosis associated with connective tissue and autoimmune diseases

For reasons that we don’t understand fully, sometimes the immune system turns against the body.

This is known as autoimmune disease. When your immune system attacks your body’s own connective tissues, they scar.

Connective tissues lie under the surface of your skin and around your internal organs and blood vessels. If autoimmune diseases, including rheumatoid arthritis, Sjögrens syndrome and scleroderma, affect your lungs, it can cause pulmonary fibrosis.

Unfortunately some of the drugs used to treat these diseases can also cause interstitial lung disease as a side effect.


It’s difficult to say what course your lung disease will take because this depends on many factors, including the particular form of autoimmune disease you have, how severe it is and the way it is affecting your lungs.

Some people live just a few years after their diagnosis. But other people survive much longer. Talk to your doctor about your individual situation.

If you or your family are facing an emotional time, call our helpline for confidential one-to-one advice and support from our dedicated team.


In addition to treatment of your lung symptoms, the best possible management of your underlying condition is essential to protect your lungs from more damage. You’ll usually be treated with immunosuppressant drugs tailored to your specific condition.

If you have concerns or need advice, call our helpline on 03000 030 555 between 9am and 5pm on a weekday or email them.

We'll take good care of your personal info and you can update the way we contact you at any time - check out our privacy policy at blf.org.uk/privacy to find out more.

Download this information (PDF, 250KB) > Order your PF information >

Last medically reviewed: August 2016. Due for review: August 2019

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.