Systemic sclerosis is an autoimmune condition. It is rare condition and more commonly affects women. It usually starts between ages 25-55 years but has been reported in children and older people.

Causes of systemic sclerosis

Autoimmune conditions occur when the body’s immune system starts to attack itself. In systemic sclerosis, the immune system targets connective tissues including blood vessels, cartilage and tendons.  Damage by the immune system causes excessive inflammation and increased production of collagen, leading to scarring or fibrosis of the body’s tissues and organs (scleroderma).  

In systemic scleroderma (systemic sclerosis) the body’s internal organs such as the lungs, heart and kidneys can be affected. If this occurs in the lungs, it results in pulmonary fibrosis.

Symptoms

People with systemic sclerosis or scleroderma often have thickening of the skin and may have difficulty swallowing. When it involves the lungs, patients may have a cough and shortness of breath and other symptoms of pulmonary fibrosis

Some patients develop pulmonary hypertension (increased blood pressure in the circulation to the lungs): symptoms include a racing or fast heartbeat, chest pain, swelling of the legs and tiredness.

Treatments

There is currently no cure for systemic sclerosis. Because it is caused by the immune system attacking your connective tissues, it is usually treated with immunosuppressive therapies.

If you have pulmonary hypertension, you may be put on specific treatments that cause the blood vessels to dilate and lower the pressure. You may also be asked to take diuretics if you have swelling of the legs.

It is essential to stop smoking as smoking can cause narrowing of the blood vessels (vasoconstriction).

Ongoing treatment

You will have regular follow-up appointments with your medical team to monitor how the condition is progressing and to discuss whether your treatment needs to change.

Outlook

Systemic sclerosis that involves the lungs can progress and is associated with a poorer outcome.

Other forms of pulmonary fibrosis

Get support

Join a support group in your area

It’s important to remember that you are not alone. There are now over 75 pulmonary fibrosis support groups around the country.

Support groups meet informally to share experiences, provide expert information and raise funds and awareness. Find a group near you.

Call our support line

APF runs an email and telephone support line offering information and advice about living with PF. Find out more.

Find us on social media

We have an active and supportive online community ready to welcome you on Facebook, Twitter and Instagram for updates, news and connections to our online community.

Watch videos of how other people cope day to day with PF.

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