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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 alter its course, but outlook varies widely and 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 variant 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 two to five years of onset. In the absence of such events or if the patient survives the initial acute progression, the disease tends to progress very slowly. It then follows a very slow course. The more severe the condition of the skin at the outset of the disease, the poorer the survival rates.

Many patients with systemic scleroderma experience a plateau in which the condition stabilizes, which is followed with a period of improvement and skin softening. No one knows why this occurs and it can occur without any association to 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.

Primary pulmonary hypertension
Pulmonary hypertension is the narrowing of the pulmonary arterioles within the lung. The narrowing of the arteries creates resistance and an increased work load for the heart. The heart becomes enlarged from pumping blood against the resistance. Some symptoms include chest pain, weakness, shortness of breath, and fatigue. The goal of treatment is control of the symptoms, although the disease usually develops into congestive heart failure.

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

  • Some patients may not even experience symptoms.
  • When it progresses, patients develop a dry cough, shortness of breath, and reduced exercise capacity.
  • 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 three years. In such cases, lung function declines rapidly over that period and then slows down.

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

The most important indication of future deterioration appears to be evidence of inflammation in the small airways (called alveolitis), which is detected using a lung test called bronchoalveolar lavage.

Pulmonary Hypertension. Pulmonary hypertension occurs in about half of 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.

It 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.
Cor pulmonale Click the icon to see an image of cor pulmonale.

Kidney Complications

Signs of kidney involvement, 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 situation is acute or chronic.

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

Renal Crisis. The most serious kidney complication is renal crisis. It is a rare event that occurs in a minority 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 progressive kidney failure. This condition may be fatal. If the condition is successfully treated, however, recurrence is rare.

Until recently, renal crisis was the most common cause of death in scleroderma. Aggressive treatment with anti-hypertensive drugs, particularly those known as angiotensin-converting enzyme (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 three 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, lower blood pressure, or lead to other complications. By damaging muscle tissue it increases the risk for heart rhythm disturbances, problems in electrical conduction, and congestive heart failure. The membrane around the heart can become inflamed causing a condition called pericarditis.

Pericarditis Click the icon to see an image of 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 gastrointestinal (GI) tract.

Complications in Upper GI Tract.

  • Gastroesophageal reflux disorder (heartburn and trouble swallowing) is a common problem and much more severe than in the general public. This develops when the scarring develops in muscles in the esophagus so that they lose motility and are unable to contract normally. There is some suggestion that patients with severe GERD may inhale 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 streak red areas from dilated 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 GI Tract. Complications in the lower tract can develop but are uncommon. They can include the following:

  • Scarring can cause obstruction and constipation. In rare cases, constipation can become so severe that the bowel becomes perforated, which can be life threatening.
  • Scarring can also impair intestinal absorption of fats (malabsorption). This can lead to bacteria proliferation that 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

Needless to say, 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% experienced at least mild pain and half of them had mild depression or greater. Depression had the greatest impact, even more than pain, in reducing their ability to function socially.

Other Complications

Other complications 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, which can be progressive and lead to severe ulcerations, 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 patients develop hypothyroidism.
Hypothyroidism Click the icon to see an image of hypothyroidism.
  • Impotence usually due to Peyronies disease (scarring of the penis) may be one of the first complications of the disease in men.
  • There 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.
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