Scleroderma, or systemic sclerosis, is a fibrosing (scarring) autoimmune disorder that can affect the skin, the lungs, the heart and the kidneys amongst other organs. The disease is due at least in part to the overproduction of cells called fibroblasts which can create scar like tissue (fibrosis) in different organs. This occurs most frequently in the lungs, the heart and the kidney- organs that are critical to bodily function. While there is no cure, there are treatments that may slow down the disease, and rheumatologists work with skin, lung, heart and kidney specialists to treat the specific organs that may be involved to help improve symptoms, limit organ damage and improve quality of life.
Causes and Risk Factors
Though the cause is unknown, scleroderma is the result of an autoimmune response. This means the symptoms are caused by the body attacking its own healthy tissues. While anyone can develop scleroderma, risk factors may include:
Gender (it tends to affect females more than males)
Age (most common between the ages of 30 to 60)
Family history of scleroderma
Some of the most common symptoms of Scleroderma include:
Tightening of the skin of the hands, feet extremities and face
Difficulty breathing due to interstitial lung disease or pulmonary hypertension
Severe hypertension which can lead to kidney failure
Diarrhea, constipation or heartburn
Red skin lesions called telangiectasia
Calcium deposits in the skin
Edema of the lower extremities
A rheumatologist can usually make the diagnosis based on an examination and evaluation of blood tests that support the diagnosis. A dermatologist can also make the diagnosis based on specific features on examination of the skin. Patients suspected of having scleroderma should also undergo the following tests to assess risk and decide if additional treatment is needed:
Pulmonary function tests, including a computerized tomography (CT) scan of the lungs
Ultrasound assessment of cardiac function
Blood and urine assessment of kidney function
While there are is no cure for scleroderma, there are treatment approaches available. Treatment for scleroderma may include:
Non-steroidal anti-inflammatory drugs (NSAIDs) to help reduce swelling and pain caused by the scleroderma.
Immunosuppressant drugs to help slow the progression of the disease.
Corticosteroids to relieve pain and minimize swelling caused by scleroderma.
Anti-hypertensives to control blood pressure and/or vasodilators to improve blood pressure.
Acid blockers for the stomach to help make swallowing easier and to minimize heartburn.
Exercise and physical therapy to help maintain flexibility and muscle strength.
Newer approaches and clinical trials are emerging to better treat patients with scleroderma.
To learn more about our services or to make an appointment with a Brigham and Women’s Hospital rheumatologist, contact one of our trained coordinators at 1-800-294-9999 to get connected with the best doctor for your needs.