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Scleroderma

Description

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

Alternative Names

Systemic sclerosis

Treatment

Because scleroderma is so variable, treatments vary depending on the patient.

The first step is to determine what form the disease has taken:

• Is it local or systemic, and if systemic, is it limited or diffuse?
• If the disease is systemic, what organs, if any, are involved?

Specific drugs are used to help combat the various mechanisms and consequences of the disease.

• Some medications keep blood vessels open (prostacylins, ACE inhibitors and others) and are used to treat Raynaud's phenomenon, heart and kidney problems, and pulmonary hypertension.
• Others, notably cyclophosphamide, reduce inflammation and block damaging immune factors. These drugs are helpful for improving skin thickness and reducing scarring, even in the lungs.
• Doctors use other treatments for specific complications, such as proton pump inhibitors for gastrointestinal problems, or light treatments for skin thickening.
• Promising investigative approaches include stem-cell transplantation.

The patient should receive treatments for specific complications as early as possible in the course of the disease, to reduce progression before irreversible hardening of tissues occurs.

Problems in Developing Treatments for Scleroderma

There is no cure for scleroderma. Experimental work is ongoing to develop procedures or to find drugs that can treat the underlying processes that cause damage. Developing effective treatments for scleroderma is very problematic, however, for the following reasons:

• Scleroderma has an extremely unpredictable progression, making it one of the most difficult rheumatic diseases to treat. It also makes drug development complicated.
• The disease, when advanced, affects many organs. Designing treatment strategies that will improve symptoms in some organs without affecting others is very difficult.
• Many drugs that are useful in other autoimmune inflammatory disorders have not proven to be very effective for scleroderma.
• Many studies do not take seasonal temperature changes into consideration. Such temperature changes are major factors in scleroderma and can distort study results.
• The disease is so uncommon that there are few patients for clinical trials. Studies, then, are very small, sometimes having only four or five patients. It is very difficult to design studies that can provide strong evidence one way or the other on treatment effects. Drugs that seem promising on small groups of patients often fail to come through on larger groups. For example, of great disappointment was the failure of relaxin (ConXn) to produce significant benefits. It had been the first drug approved specifically for scleroderma.

Treating the Whole Patient

The course of scleroderma is difficult to predict. The disease can evolve slowly over time with few symptoms, or progress rapidly and become very severe. The patient, then, must live with considerable uncertainty and emotional stress. Support associations, non-medical aids to help relieve symptoms, and other life-style measures can be extremely important and helpful.

• 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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