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Scleroderma is an autoimmune disease affecting the skin and other organs of the body, meaning that the body’s immune system is causing inflammation and other abnormalities in these tissues. The main finding in scleroderma is thickening and tightening of the skin and inflammation and scarring of many body parts, leading to problems in the lungs, kidneys, heart, intestinal system and other areas. There is still no cure for scleroderma but effective treatments for some forms of the disease are available.
Scleroderma is relatively rare. About 75,000 to 100,000 people in the U.S. have this disease; most are women between the ages of 30 and 50. Twins and family members of those with scleroderma or other autoimmune connective tissue diseases, such as lupus, may have a slightly higher risk of getting scleroderma. Children can also develop scleroderma, but the disease is different in children than in adults.
Although the underlying cause is unknown, promising research is shedding light on the relationship between the immune system and scleroderma. A great deal of research is also underway to find better treatments for scleroderma and, hopefully, someday a cure.
Scleroderma (also known as systemic sclerosis) is a chronic disease that causes the skin to become thick and hard, a buildup of scar tissue, and damage to internal organs such as the heart and blood vessels, lungs, stomach and kidneys. The effects of scleroderma vary widely and range from minor to life-threatening, depending on how widespread the disease is and which parts of the body are affected.
The two main types of scleroderma are:
Localized scleroderma usually affects only the skin, although it can spread to the muscles, joints and bones. It does not affect internal organs. Symptoms include discolored patches on the skin (a condition called morphea); or streaks or bands of thick, hard skin on the arms and legs (called linear scleroderma). When linear scleroderma occurs on the face and forehead, it is called en coup de sabre.
Systemic scleroderma is the most serious form of the disease, can affect the skin, muscles, joints, blood vessels, lungs, kidneys, heart and other organs. There are two major forms of systemic scleroderma: limited cutaneous systemic sclerosis (also called CREST syndrome) and diffuse cutaneous systemic scleroderma.
The cause of scleroderma is not known. Genetic factors (different genes) appear be important in the disease. Although exposure to certain chemicals may play a role in some people having scleroderma, the vast majority of patients with scleroderma do not have a history of exposure to any suspicious toxins. The cause of scleroderma is likely quite complicated.
Diagnosis can be tricky because symptoms may be similar to those of other diseases. There is not a blood test that can say for sure that you have scleroderma, although numerous antibodies have been associated with this condition. To make a diagnosis, a doctor will ask about the patient’s medical history, do a physical exam and possibly order lab tests and X-rays. Careful clinical evaluation is the primary method for monitoring scleroderma. X-rays and computerized tomography (CT) scans are used to look at bone abnormalities. Thermography can detect differences in skin temperature between the lesion and normal tissue. Ultrasound and magnetic resonance imaging (MRI) can aid soft tissue assessment. Some symptoms he or she will look for include:
While some treatments are effective in treating some aspects of this disease, there is no drug that has been clearly proven to stop, or reverse, the key symptom of skin thickening and hardening. Medications that have proven helpful in treating other autoimmune diseases, such as rheumatoid arthritis and lupus, usually don’t work for people with scleroderma. Doctors aim to curb individual symptoms and prevent further complications with a combination of drugs and self-care. For example:
There are two types of lung disease that patients with scleroderma may develop. The first type is called interstitial lung disease, which causes scarring of the lung tissue. There is evidence that medications, such as cyclophosphamide and mycophenolate, are somewhat effective in treating the interstitial lung disease in scleroderma. Clinical trials are ongoing, evaluating the effectiveness of several other drugs for this problem. The second type of lung disease seen in scleroderma is pulmonary arterial hypertension (high blood pressure in the arteries in the lungs). A number of drugs have become available to treat this condition, which work by opening up the blood vessels in the lungs to improve flow. Medications used for treatment of pulmonary hypertension are usually managed by specialists in the condition, and include prostacyclin-like drugs (epoprostenol, treprostinol, iloprost), the endothelin receptor antagonists (bosentan, ambrisentan), and PDE-5 inhibitors (sildenafil, vardenafil, tadalafil).
Much research is ongoing into new treatments for scleroderma. Patients and their families should know that experts remain optimistic and take comfort in the fact that work towards a cure will continue.
Scleroderma can involve almost every organ system in the body. Although symptoms vary greatly from patient to patient, it can dramatically impact someone’s life. Patients should consult a rheumatologist—or a team of specialists---who are experienced in dealing with this complicated disease. Several other diseases that affect the skin are sometimes confused with scleroderma.
Living with scleroderma is quite challenging. Everyday activities can sometimes be difficult due to physical limitations and pain. Problems with digestion may require changes in diet; patients often have to eat several small meals rather than fewer large meals. Patients must also keep the skin well-moisturized to lessen stiffness and be careful during activities such as gardening, cooking—even opening envelopes---to avoid finger injuries. To keep the body warm, patients should dress in layers; wear socks, boots and gloves; and avoid very cold rooms. Unfortunately, moving to a warmer climate does not necessarily lead to dramatic improvement. Exercise and/or physical therapy may ease stiffness in the joints.
Patients must also deal with the psychological setbacks that come from living with a disease that is chronic, uncommon and currently incurable. Because scleroderma can cause significant changes in appearance, a patient’s self-esteem and self-image are almost always affected. The support of family and friends is vital in helping to maintain a good quality of life.
Updated March 2017 by Paul Sufka, MD and reviewed by the American College of Rheumatology Committee on Communications and Marketing.
This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical or health condition.
© 2017 American College of Rheumatology
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