Connective tissue and autoimmune diseases

This page is about pulmonary fibrosis caused by connective tissue and autoimmune diseases.

What is pulmonary fibrosis caused by connective tissue and autoimmune diseases?

For reasons 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 cause scarring (fibrosis).

Connective tissues lie under the surface of your skin and around your internal organs and blood vessels. If an autoimmune disease, including rheumatoid arthritis, Sjögrens syndrome and systemic sclerosis (scleroderma), affects your lungs, they can cause pulmonary fibrosis (scarring of the lungs). This is sometimes called a connective tissue disease related ILD or CT-ILD.

Unfortunately, some of the drugs used to treat these autoimmune diseases may rarely also cause lung damage as a side effect.

The tendency to develop some forms of auto-immune or connective tissue diseases is genetic. If you have this type of pulmonary fibrosis, your doctor should ask about your family history of these as well as other lung diseases.

You can read more about rheumatoid arthritis ILD and systemic sclerosis (scleroderma) on the Action for Pulmonary Fibrosis website.

What’s the outlook of pulmonary fibrosis caused by autoimmune disease?

Your prognosis (likely course of your condition) 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 live much longer. Talk to your doctor about your individual situation.

What’s the treatment for pulmonary fibrosis caused by autoimmune disease?

You might need to be under the care of both a rheumatologist and a respiratory specialist. You’ll usually be treated with immunosuppressant drugs.

An antifibrotic drug – called Nintedanib – has also been shown to help slow the progression of pulmonary fibrosis, in people where the fibrosis is progressing despite immunosuppressant drugs. Nintedanib might be recommended as well as immunosuppressant drugs, or sometimes instead of, depending on the individual.

As well as treating lung symptoms, managing your underlying condition is essential to protect your lungs from more damage.

Read more about treating pulmonary fibrosis and ways you can look after yourself.

Help to stop smoking

If you smoke, stopping is very important for your lung condition and overall health. Your GP can refer you to free help to stop smoking.

Did you find this information useful?

We use your comments to improve our information. We cannot reply to comments left on this form. If you have health concerns or need clinical advice, call our helpline on 0300 222 5800 between 9am and 5pm on a weekday or email them.

Page last reviewed:
Next review due: