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Scleroderma

Description

An in-depth report on the causes, diagnosis, treatment, and prevention of scleroderma.

Alternative Names

Systemic sclerosis

Prognosis

At this time there is no cure for scleroderma and no treatment to change its course, but outlook varies widely. Many patients, even many with systemic scleroderma, can expect a normal lifespan.

General Outlook of Localized Scleroderma. Localized scleroderma nearly always carries a good prognosis and a normal life span. Even localized scleroderma, however, can cause some severe effects in children, including impaired growth, limb imbalance, and problems in flexing and bending muscles.

General Outlook of Systemic Scleroderma

• Limited Scleroderma. Patients with limited CREST scleroderma can usually expect a favorable outlook and normal lifespan if the disease affects only the hands and face. The course of this type of scleroderma still tends to be slowly progressive and, in some cases, may affect internal organs.
• Diffuse Scleroderma. The severity of diffuse scleroderma varies widely, and it is very difficult to predict its course. It generally follows one of two paths: If it is acute or rapidly progressing, it may be a life-threatening condition that affects internal organs. The most critical period for rapid progression is usually within the first 2 - 5 years of the start of the disease. In the absence of rapid progression, or if the patient survives the initial acute progression, the disease tends to progress very slowly. The more severe the condition of the skin is at the start of the disease, the poorer the survival rates.

Many patients with systemic scleroderma experience a plateau in which the condition stabilizes. This plateau is followed by a period of improvement and skin softening. No one knows why this occurs, and it can happen regardless of treatment. In one study, patients with systemic scleroderma who experienced such improvements also had better survival rates (80% at 10 years) than those whose skin did not improve (60% 10-year survival rate).

Lung Complications

Lung problems are usually the most serious complications of systemic scleroderma. They are now the leading cause of death in scleroderma patients. Two major lung conditions associated with scleroderma, pulmonary fibrosis and pulmonary hypertension, can occur either together or independently.

Pulmonary Fibrosis. Scleroderma in the lung causes scarring (pulmonary fibrosis). Pulmonary fibrosis occurs in up to 80% of scleroderma patients, although the progression is very slow and patients have with a wide range of symptoms:

• Some patients may not experience any symptoms.
• When pulmonary fibrosis progresses, patients develop a dry cough, shortness of breath, and reduced exercise ability.
• Severe pulmonary fibrosis occurs in about 16% of patients with diffuse scleroderma. About half of these patients experience the most profound changes within the first 3 years. In such cases, lung function gets worse rapidly over that period, and then the progression slows down.

One of the most serious complications of pulmonary fibrosis is interstitial lung disease, which causes breathing difficulties and a decline in lung function. This condition also places the patient at higher risk for lung cancer. One study suggested that interstitial lung disease may be due to severe dysfunction in the esophagus, which causes patients to breathe in tiny amounts of stomach acid.

The most important indication of future worsening in the lungs appears to be evidence of inflammation in the small airways (alveolitis). Doctors detect alveolitis by using a lung test called bronchoalveolar lavage.

Pulmonary Hypertension. Pulmonary hypertension occurs in about half of scleroderma patients. In this condition, blood pressure in the lungs increases, in some cases to a dangerous level. The primary symptom is shortness of breath, which is often severe.

Pulmonary hypertension can develop in one of two ways:

• As a complication of pulmonary fibrosis
• As a direct outcome of the scleroderma process itself. In this case, it is most likely to develop in patients with limited scleroderma after many years.

Pulmonary hypertension can be very serious in the short- and long-term.

• If pulmonary hypertension develops suddenly it can cause respiratory failure, which is life threatening.
• Over time, pulmonary hypertension may cause a condition called cor pulmonale , in which the right side of the heart increases in size. In some cases, this enlargement can lead to heart failure.

Kidney Complications

Signs of kidney problems, such as increased levels of protein in the urine and mild hypertension, are common in scleroderma. As with pulmonary hypertension, the degree of severity depends on whether the kidney problems are acute or chronic.

Slow Progression. The typical course of scleroderma in the kidney is a slow progression that may produce some damage. Such damage does not usually require dialysis.

Renal Crisis. The most serious kidney complication in scleroderma is renal crisis. It is a rare event that occurs in a small number of patients with diffuse scleroderma, most often early in the course of the disease. This syndrome includes a life-threatening condition called malignant hypertension, a sudden increase in blood pressure that can cause rapidly advancing kidney failure. This condition may be fatal. However, if the condition is successfully treated, recurrence is rare.

Until recently, renal crisis was the most common cause of death in scleroderma. Aggressive treatment with drugs that lower blood pressure, particularly those known as ACE inhibitors, is proving to be successful in reducing this risk.

Heart Complications

Many patients with even limited scleroderma have some sort of functional heart problem, although severe complications are uncommon and occur in only about 15% of patients with diffuse scleroderma. As with other serious organ complications, they are more likely to occur within 3 years of the onset of the disease.

Fibrosis of the Heart. The most direct effect that scleroderma has on the heart is fibrosis (scarring). It may be very mild or it can cause pain, low blood pressure, or other complications. By damaging muscle tissue, the scarring increases the risk for heart rhythm problems, problems in electrical conduction, and heart failure. The membrane around the heart can become inflamed, causing a condition called pericarditis.

Effects of Pulmonary Hypertension. Pulmonary hypertension and kidney problems associated with scleroderma can also affect the heart.

Gastrointestinal Complications

The following complications may occur in the digestive tract:

Complications in the Upper Digestive Tract.

• Gastroesophageal reflux disorder (heartburn and trouble swallowing) is a common problem and much more severe than in the general public. This disorder, also known as GERD, develops when scarring in muscles of the esophagus causes them to lose the ability to contract normally. Some experts believe that patients with severe GERD may breathe in microscopic amounts of stomach acid, which in turn may be a major cause of lung scarring.
• About 80% of patients also experience impaired stomach activity, with a delay in stomach emptying being very common.
• Some patients develop "watermelon stomach" (medically referred to as CAVE syndrome), in which the stomach develops red streaked areas from widened blood vessels. This causes a slow bleeding that can cause anemia over time.
• There may be a higher risk for stomach cancer.

Complications in the Lower Digestive Tract. Complications in the lower tract can develop but are uncommon. They can include the following:

• Scarring can cause blockage and constipation. In rare cases, constipation can become so severe that the bowel develops holes or tears, which can be life threatening.
• Scarring can also damage the absorption of fats in the intestines. This can lead to an increase in the number of bacteria, which causes watery diarrhea.
• Fecal incontinence (the inability to control bowel movements) may be more common than studies indicate, since patients are reluctant to report it.

Many patients, however, have few or even no lower gastrointestinal symptoms.

Impact on Quality of Life

The many complications of scleroderma can have a major impact on the person's sense of well-being. Patients are greatly concerned about changes in their appearance, particularly alterations caused by tightening of the facial skin. A 2002 study on scleroderma patients reported that 63% of scleroderma patients experienced at least mild pain, and half of them had some degree of depression. Depression had the greatest impact, even more than pain, in reducing patients' ability to function socially.

Other Complications

Other complications of scleroderma may include the following:

• Patients with CREST may be at increased risk for biliary cirrhosis, an inflammatory autoimmune disorder of the liver.
• Nerve damage may occur in the extremities (legs and feet, arms and fingers), causing numbness and pain. This damage can be progressive and lead to severe ulcerations (open sores), particularly in the hands. The feet are less often affected, but when they are, the disease tends to affect the joints and cause pain.
• Bone loss (osteoporosis) can occur because of impaired blood flow.
• About half of the patients develop hypothyroidism.

• Impotence, usually due to scarring of the penis, may be one of the first complications of the disease in men.
• There is some evidence of changes in the elastic properties of large arteries, such as those leading into the legs and up to the brain, in patients with scleroderma. Such changes theoretically increase the risk for stroke. Current evidence does not reveal an elevated risk.
• Some studies, using imaging techniques, have found changes in brain tissue, but because the brain has little connective tissue, scleroderma appears to have little effect on mental functioning, except possibly in late stages of severe disease.
• Systemic scleroderma does not generally affect fertility in women. Pregnant women with scleroderma, however, have a slightly increased risk of premature birth and low birth weight babies.

• Review Date: 12/14/2006
• Reviewed By: Harvey Simon, MD, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital

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