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Scleroderma

Description

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

Alternative Names

Systemic sclerosis

Medications

Drugs that relax and open blood vessels (vasodilators) have been a mainstay for treating and preventing complications in scleroderma. As more is known about the disease, however, additional new drugs are used to treat this difficult disease. Some of these drugs affect blood clotting and smooth muscles in the blood vessels.

Vasodilators. Vasodilators have been key medicines in the treatment of scleroderma. They relax and open blood vessels, and are important for treating most of the symptoms and complications of scleroderma. [These agents are also discussed under many of the sections covering complication of treatments.]

Calcium-Channel Blockers. Calcium-channel blockers are the standard vasodilating agents. Short- or sustained-release nifedipine (Adalat, Procardia) is the gold standard. Others used include diltiazem (Cardizem, Dilacor). Side effects vary among different preparations, and may include fluid accumulation in the feet, constipation, fatigue, impotence, gingivitis, flushing, and allergic symptoms. Grapefruit juice appears to boost the effects of these drugs.

• Nitrates. Nitrates relax smooth muscles and therefore open arteries. They are available as topical or in a form taken by mouth. Side effects of nitrates include headaches, dizziness, nausea, blurred vision, fast heartbeat, and sweating. Lying down with the legs elevated can relieve low blood pressure and dizziness. Alcohol, beta blockers, calcium-channel blockers, and certain antidepressants can significantly worsen these side effects. Withdrawal from nitrates should be gradual. Some severe reactions have occurred when people have stopped abruptly.

Prostacyclins (also called Prostaglandins). Prostacyclins open blood vessels and also have anti-blood clotting properties. Specific agents, such as iloprost, appear to reduce levels of connective tissue growth factor, a molecule important in the abnormal production of cells that cause collagen buildup. A 2005 study reported that prostavasin, a drug related to prostacyclins, improved circulation to the hands and feet. Several prostacyclins are being used for scleroderma, although none have been approved specifically for the condition. Iloprost has been studied the longest. Other promising prostacyclins or similar drugs include alprostadil (prostaglandin E1), epoprostenol (Flolan,), and treprostinil (Remodulin).

Endothelin Receptor Antagonists. Bosentan (Tracleer) is a drug taken by mouth. It is called an endothelin receptor antagonist. It controls endothelin, a powerful molecule that causes blood vessels to narrow. It improves blood flow and is becoming important for treating patients with scleroderma, especially for preventing finger ulcers and improving hand function. Although experts initially thought bosentan might help scleroderma patients with pulmonary hypertension, a 2005 study concluded that the drug did not work for such patients. In fact, the researchers found that bosentan might even make the condition worse.

ACE Inhibitors and Similar Agents for High Blood Pressure and Renal Crises

The most effective approach at this time for preventing renal crises is to institute aggressive blood pressure-lowering treatment before blood tests show kidney damage.

Angiotensin Converting Enzyme (ACE) Inhibitors. Many medications are available for controlling blood pressure, but ACE inhibitors appear to be the most effective for scleroderma patients, because of their protective actions in the kidney. ACE inhibitors include captopril (Capoten), enalapril (Vasotec), quinapril (Accupril), benazepril, and lisinopril (Prinivil, Zestril). Side effects are uncommon but may include an irritating cough, large drops in blood pressure, and allergic reactions. The drug picotamide can help reduce the frequency of coughs.

One rare but severe side effect, granulocytopenia, has been observed. This extreme reduction in white blood cells can be minimized with lower medication dosages. There has been some concern that ACE inhibitors may impair lung function, but studies to date have been reassuring.

Angiotensin II Receptor Antagonists. Angiotensin II receptor antagonists (losartan, candesartan cilexetil, and valsartan) have benefits similar to ACE inhibitors and may have fewer or less severe side effects, including coughing. They may also have positive effects on blood vessels. Small studies showing improvement in Raynaud's phenomenon warrant further research.

Immunosuppressive Treatments

One major approach to scleroderma is to use treatments that suppress the immune system, and therefore reduce the activity of the harmful processes leading to scleroderma. Such treatments are used effectively in other autoimmune diseases. Their use in scleroderma varies depending on the location and severity of the disease process.

An important 2002 study employed an approach called high-dose immunosuppressive therapy, which uses radiation, powerful immunosuppressant drugs, and other therapies to strongly suppress the immune system. This is a very toxic treatment, but improvements in skin and other indicators of scleroderma were more significant than those reported with other therapies. More research is needed.

Cyclophosphamide (Cytoxan) is the most important immunosuppressant currently used for scleroderma. A small study found that patients with scleroderma-related lung disease respond better to intravenous cyclophosphamide than those without such lung disease. Additionally, results from the Scleroderma Lung Study show that cyclophosphamide improves the lung condition of scleroderma patients with lung complications. Patients took a daily dose of cyclophosphamide, by mouth, for a full year. The improvements in energy levels and breathing lasted for 2 years (the duration of the study). When used with stem cell transplantation, high doses of cyclophosphamide are proving to be safe for patients with systemic sclerosis.

Other drugs used to suppress the immune system may be useful in specific cases. They include D-penicillamine (which may be useful for skin symptoms), methotrexate (Rheumatrex), corticosteroids, cyclosporine (Sandimmune, Neoral), and chlorambucil (Leukeran). All of these agents have potentially severe side effects.

• 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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