Pulmonary fibrosis

Pulmonary fibrosis associated with connective tissue and autoimmune diseases

For reasons that we don’t fully understand, 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, they can cause pulmonary fibrosis. Unfortunately some of the drugs used to treat these diseases can also cause interstitial lung disease as a side effect.

The tendency to develop some forms of auto-immune or connective tissue diseases is genetic so your doctor should ask about your family history of these as well as other lung diseases.

Symptoms

The course your lung disease takes will depend on many factors, including the particular form of autoimmune disease you have, how severe it is and the way it affects your lungs. Some people live just a few years after their diagnosis, particularly if they develop complications such as pulmonary hypertension. But other people survive much longer. Talk to your doctor about your individual situation.

Treatment

You might need to be under the care of both a rheumatologist and respiratory specialist. You’ll usually be treated with immunosuppressant drugs. As well as treating lung symptoms, managing your underlying condition is essential to protect your lungs from more damage

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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.