DescriptionAn in-depth report on the causes, diagnosis, treatment, and prevention of scleroderma.
Alternative NamesSystemic Sclerosis
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
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 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 patients, although the progression is very slow and patients respond with a wide range of symptoms:
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:
Pulmonary hypertension can be very serious in the short- and long-term.
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.
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.
Effects of Pulmonary Hypertension. Pulmonary hypertension and kidney problems associated with scleroderma can also affect the heart.
The following complications may occur in the gastrointestinal (GI) tract.
Complications in Upper GI Tract.
Complications in the Lower GI Tract. Complications in the lower tract can develop but are uncommon. They can include the following:
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 may include the following: