Scleroderma (cont.)

How is scleroderma diagnosed?

The diagnosis of the scleroderma syndrome is based on the finding of the clinical features of the illnesses. Nearly all patients with scleroderma have blood tests which suggest autoimmunity, antinuclear antibodies (ANAs). A particular antibody, the anticentromere antibody, is found almost exclusively in the limited, or CREST, form of scleroderma. Anti-Scl 70 antibody (antitopoisomerase I antibody) is most often seen in patients with the diffuse form of scleroderma.

Other tests are used to evaluate the presence or extent of any internal disease. These may include upper and lower gastrointestinal tests to evaluate the bowels, chest x-rays, lung function testing, and CAT scanning to examine the lungs, EKG and echocardiograms, and sometimes heart catheterization to evaluate the pressure in the arteries of the heart and lungs.

How is scleroderma treated?

Treatment of scleroderma is directed toward the individual feature(s) affecting different areas of the body.

Aggressive treatment of elevations in blood pressure have been extremely important in prevent kidney failure. Blood-pressure medications, such as captopril, are frequently used.

Recent data indicate that colchicine can be helpful in decreasing the inflammation and tenderness that periodically accompanies the calcinosis nodules in the skin. Skin itching can be relieved with lotions (emollients) such as Eucerin and Lubriderm.

Mild Raynaud's phenomenon may require only hand warming and protection. Low-dose aspirin is often added to prevent tiny blood clots in the fingers, especially in patients with a history of fingertip ulcerations. Moderate Raynaud's phenomenon can be helped by medications that open up the arteries, such as nifedipine (Procardia, Adalat) and nicardipine (Cardene), or with topical nitroglycerin applied to the most affected digit (most effective on the sides of the digit where the arteries are). Gently applied finger splinting can protect tender tissues. A class of medications that is typically used for depression, called serotonin reuptake inhibitors, such as fluoxetine (Prozac), can sometimes improve the circulation of the affected digit. Severe Raynaud's phenomenon can require surgical procedures, such as those to interrupt the nerves of the finger that stimulate constriction of the blood vessels (digital sympathectomy). Ulcerations of the fingers can require topical or oral antibiotics.

Esophagus irritation and heartburn can be relieved with omeprazole (Prilosec), esomeprazole (Nexium), or lansoprazole (Prevacid). Antacids can also be helpful. Elevating the head of the bed can reduce the back-flow of acid into the esophagus that causes inflammation and heartburn. Avoiding caffeine and cigarette smoking also helps.

Constipation, cramping, and diarrhea is sometimes caused by bacteria that can be treated with tetracycline or erythromycin. Recent studies have shown that erythromycin could also be used. Increased fluid intake and fiber intake are good general measures.

Irritated, itchy dry skin can be helped by emollients such as Lubriderm, Eucerin, or Bagbalm.

Telangiectasias, such as those on the face, can be treated with local laser therapy. Sun exposure should be minimized as it can worsen telangiectasias.

Approximately 10% of patients with the CREST variant develop elevated pressures in the blood vessels to the lungs (pulmonary hypertension). Abnormally elevated blood pressure of the arteries supplying the lungs is often treated with calcium antagonist medications, such as nifedipine, and blood-thinning drugs (anticoagulation). More severe pulmonary hypertension can be helped by continuous intravenous infusion of prostacyclin (Iloprost). A new drug taken by mouth, bosentan (Tracleer), is now available to treat severe pulmonary hypertension.

Additionally, medications are used to suppress the overly active immune system that seems to be spontaneously causing the disease in organs affected. Medications used for this purpose include penicillamine, azathioprine, and methotrexate. (Recent research has found that low-dose penicillamine [Depen, Cuprimine] [125mg every other day] is as effective as previously used high doses of penicillamine, with less toxicity.) Serious inflammation of the lungs (alveolitis) can require immune suppression with cyclophosphamide (Cytoxan) along with prednisone. The optimal treatment of scleroderma lung disease is an area of active research. Stem-cell transplantation is being explored as a possible option.

No medication has been found to be universally effective for all patients with scleroderma. In an individual patient, the illness may be mild and not require treatments. In some, the disease is ravaging and relentless.

Next: What is the outlook (prognosis) for patients with scleroderma? »

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