Scleroderma is a disorder that causes the body to make too much collagen, the result being inflammation (irritation) and stiffness. Collagen is the main protein in connective tissue, which supports the organs and skin. Too much collagen causes skin and organs to get thick and stiff. Scleroderma can affect the heart, esophagus (tube connecting the mouth and stomach), kidneys, lungs, blood vessels, and skin.
Scleroderma affects women four times more often than men, usually starting at age 30 to 50.
The cause is unknown, but scleroderma is an autoimmune disorder. In autoimmune disorders, the body’s own immune system mistakenly attacks normal parts of the body and causes disease.
People can have many symptoms. Mild to severe symptoms depend on the organs affected. Most common symptoms are dry eyes and mouth, joint stiffness and pain, heartburn, muscle pain, numbness, poor circulation, poor wound healing, swallowing problems, weakness, and weight loss. Skin becomes thick, hard, and tight and loses flexibility. The face looks masklike, with thin lips and furrowing around the mouth. Fingers become thick, stiff, painful, and less moveable.
Complications include kidney failure (most common cause of death), heart failure, abnormal heart rhythms, high blood pressure, poor lung function, heartburn, and constipation.
The health care provider will take a medical history, do an examination, order blood and urine tests, do electrocardiography (ECG), and x-rays. The ECG measures the heart’s electrical activity. A small piece of skin may be taken and looked at with a microscope. Since scleroderma affects many organs, your primary care health care provider may refer you to a rheumatologist (specialist of muscle joint diseases), a nephrologist (kidney specialist), a dermatologist (skin specialist), a cardiologist (heart specialist), a pulmonologist (lung specialist), and a gastroenterologist (specialist of digestive tract).
Scleroderma isn’t curable. Treatment is aimed at controlling symptoms, relieving symptoms, and treating or preventing complications. Treatment consists of general measures and medicines. General measures include medications for heartburn or gastroesophageal reflux; avoiding smoking, alcohol, and caffeine; and not eating close to bedtime or lying down. For joint pain and stiffness, use mild heat. Avoid very cold temperatures and skin injuries and burns such as from hot water. Psychological counseling and biofeedback may help depression. Physical therapy and occupational therapy improve joint mobility.
Helpful medicines include steroids, over-the-counter antacids or prescription drugs (gastroesophageal reflux), blood pressure drugs, aspirin or anti-inflammatory drugs (arthritis), and immunomodulators (which help the immune system). Lotions, moisturizers, and bath oils soften the skin.
Contact the following sources:
Copyright © 2016 by Saunders, an imprint of Elsevier, Inc.
Ferri’s Netter Patient Advisor