Scleroderma, or systemic sclerosis, is a chronic connective tissue disease generally classified as one of the autoimmune rheumatic diseases.

The word “scleroderma” comes from two Greek words: “sclero” meaning hard, and “derma” meaning skin. Hardening of the skin is one of the most visible manifestations of the disease. The disease has been called “progressive systemic sclerosis,” but the use of that term has been discouraged since it has been found that scleroderma is not necessarily progressive. The disease may take several forms which will be explained later. There is also much variability among patients.

What scleroderma is not

Scleroderma is not contagious, it is not infectious, it is not cancerous or malignant.

How serious is scleroderma?

Any chronic disease can be serious. The symptoms of scleroderma vary greatly from individual to individual, and the effects of scleroderma can range from very mild to life-threatening. The seriousness will depend on what parts of the body are affected and the extent to which they are affected. A mild case can become more serious if not properly treated. Prompt and proper diagnosis and treatment by qualified physicians may minimize the symptoms of scleroderma and lessen the chance for irreversible damage.

How is scleroderma diagnosed?

The diagnostic process may require consultation with rheumatologists (arthritis specialists), and/or dermatologists (skin specialists) and require blood studies and numerous other specialized tests depending upon which organs are affected.

Who develops scleroderma, and when?

There are an estimated 300,000 people in the United States who have scleroderma, about one third of whom have the systemic form of scleroderma. Since scleroderma presents with symptoms similar to other autoimmune diseases, diagnosis is difficult and there may be many misdiagnosed or undiagnosed cases as well.

Localized scleroderma is more common in children, whereas systemic scleroderma is more common in adults. Overall female patients outnumber male patients about four to one, and the average age at diagnosis is in the forties.

Factors other than sex, such as race and ethnic background, may influence the risk of getting scleroderma, the age of onset, and the pattern or severity of internal organ involvement. The reasons for this are not clear. Although scleroderma is not directly inherited, some scientists feel there is a slight predisposition to it in families with a history of rheumatic diseases.

However, scleroderma can develop and is found in every age group from infants to the elderly, but its onset is most frequent between the ages of 25 to 55. When doctors say "usually" or "for the most part," the reader should understand that variations frequently occur. Many patients get alarmed when they read medical information that seems to contradict their own experiences, and conclude that what has happened to them is not supposed to happen. There are many exceptions to the rules in scleroderma, perhaps more so than in other diseases. Each case is different, and information should be discussed with your own doctor.

What causes scleroderma?

The exact cause or causes of scleroderma are still unknown, but scientists and medical investigators in a wide variety of fields are working hard to make those determinations. It is known that scleroderma involves overproduction of collagen.

Is scleroderma genetic?

Most patients do not have any relatives with scleroderma and their children do not get scleroderma. Research indicates that there is a susceptibility gene which raises the likelihood of getting scleroderma, but by itself does not cause the disease.

What is the treatment for scleroderma?

At the present time, there is no cure for scleroderma, but there are many treatments available. Some are directed at particular symptoms like heartburn, which can be controlled by medications called proton pump inhibitors or medicine to improve the motion of the bowel. Some treatments are directed at decreasing the activity of the immune system. Some people with mild disease may not need medication at all and occasionally people can go off treatment when their scleroderma is no longer active. Because there is so much variation from one person to another there is great variation in the treatments prescribed.

http://www.scleroderma.org/medical/overview.shtm