Progressive Systemic Sclerosis - Scleroderma

Scleroderma is a widespread autoimmune disorder. It causes the skin and other body parts to slowly degenerate, thicken, and stiffen. An autoimmune disorder is one in which the body produces antibodies against its own tissues for no known reason.

What is going on in the body?

Scleroderma results from an overproduction of collagen, which is the main supportive protein in the body. The excess collagen affects tiny blood vessels and almost every organ.

A person may develop either a localized or a systemic form of scleroderma. The localized form is most common in children. It usually involves a few places on the skin, soft tissues, or muscles. The localized form rarely, if ever, progresses to the systemic form of scleroderma.

The systemic form affects skin, muscles, and soft tissues. It also affects other body organs and structures, such as the following:

What are the causes and risks of the condition?

The exact cause of scleroderma is unknown. It is generally thought to be an autoimmune disorder.

New research findings suggest that autoimmune disorders may be triggered by a transfer of cells between the fetus and the mother during pregnancy. Women with scleroderma have more fetal cells in their blood decades after a pregnancy than women who don't have scleroderma. While further research is needed to substantiate these findings, the study does offer an explanation for the much higher incidence of autoimmune disorders in women than in men.

People heavily exposed to certain chemicals or substances may be at higher than normal risk for scleroderma. These substances include the following:

Mild cases may develop from occupational repetitive stresses on the hand, such as from working with a jackhammer.

This condition usually occurs between the ages of 30 and 50, but it may also occur in children. It is four times more common in women than men but can affect either sex.

What are the signs and symptoms of the condition?

Scleroderma can progress slowly, over many years. It often starts with the appearance of Raynaud's phenomenon. This condition causes abnormal sensitivity to cold in the fingers and toes. Swelling might be noted in the hands.

Sweat secretion is decreased over involved areas of skin. The skin becomes increasingly hard and dry. Extremities stiffen, and mobility is decreased. The mouth can become rigid. The face may look masklike and immobile. Body image can be negatively affected. Changes not visible inside the body are important and threatening.

Following are some additional signs and symptoms of scleroderma:

How is the condition diagnosed?

The diagnosis of scleroderma begins with a medical history and physical exam. Raynaud's phenomenon may be an early sign of scleroderma.

A skin biopsy may help diagnose scleroderma. A tissue sample of the affected skin is removed for laboratory study. The provider may also order tests of heart, lung, and GI tract function.

What can be done to prevent the condition?

There is no known way to prevent scleroderma.

What are the long-term effects of the condition?

There is no cure for scleroderma at this time. In a minority of people, problems involving the organs can cause:

Death may result from gastrointestinal, heart, kidney, or lung involvement.

What are the risks to others?

Scleroderma is not contagious and poses no risk to others.

What are the treatments for the condition?

Treatment depends on the symptoms and severity of the scleroderma. Following is a list of some of the medications used to treat this disorder:

Psychotherapy or counseling may help the person adjust to living with an incurable disease. There are support groups for this disorder. Joining one may help the person to deal with the illness.

What are the side effects of the treatments?

Medications used to treat scleroderma have many side effects. The side effects include the following:

Calcium channel blockers have long been used to treat scleroderma as well as high blood pressure. However, the findings of two recent studies show that people who take a calcium channel blocker have a much higher incidence of complications than people taking other medications for high blood pressure. The findings of one study, for example, showed that the risk of heart attack was 27% greater. The risk of congestive heart failure was 26% higher in people taking calcium channel blockers. The American Heart Association recommends discussing the risks and benefits of the medication with a healthcare provider.

What happens after treatment for the condition?

The course of scleroderma varies unpredictably. A person who only has the local form of scleroderma has a better prognosis. An individual with severe systemic scleroderma may have chronic lung, kidney, gastrointestinal, and cardiovascular problems.

How is the condition monitored?

A person with scleroderma should have frequent physical exams by his or her healthcare provider. This helps the provider monitor the activity of the disorder and determine possible complications. Frequent blood tests may help monitor the disorder as well. Any new or worsening symptoms should be reported to the healthcare provider.

Author:Gail Hendrickson, RN, BS
Date Written:
Editor:Ballenberg, Sally, BS
Edit Date:03/30/01
Reviewer:Barbara Mallari, RN, BSN, PHN
Date Reviewed:08/06/01

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