Scleroderma

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Treatment

There is no cure for systemic sclerosis and the goals of treatment are to relieve symptoms and maintain function. Most treatments are used for a particular symptom or the particular organ involved. It is important for patients with scleroderma to be involved in treatment decisions. Patients with systemic sclerosis should not smoke.

Primary treatment for skin involvement is to apply moisturizers (e.g., topical steroids and emollients). Psoralen and ultraviolet A (PUVA) therapy may also be used. This treatment involves exposing the patient to increasing amounts of ultraviolet A (UVA) rays with an oral or topical substance called psoralen, which makes the skin more sensitive to treatment and improves its effectiveness.

If sores (ulcers) develop on the skin, antibiotic ointments may be useful. Calcinosis (abnormal calcium deposits in the skin) can be treated using medications such as colchicine (used to treat gout), anticoagulants (prevent blood clotting), steroids (e.g., prednisone), and calcium channel blockers (used to treat heart conditions). In severe cases, surgical or laser removal of the deposits can be performed.

Raynaud's syndrome can be treated by avoiding stress and cold temperatures (dressing in layers) and using medications such as calcium channel blockers and vasodilating drugs, which increase blood flow.

Joint and muscle pain and stiffness may be treated using nonsteroidal anti-inflammatories (NSAIDs; e.g., ibuprofen), aspirin, and, in severe cases narcotic pain relievers. Angiotensin-converting enzyme (ACE) inhibitors (e.g., Captopril®) may be used to treat kidney involvement. In severe cases, dialysis or kidney (renal) transplant may be necessary.

In some cases, gastrointestinal involvement can be treated using conservative measures, such as elevating the head of the bed, decreasing caffeine intake, consuming smaller meals. Antacids may also be helpful. Proton pump inhibitors (e.g., omeprazole [Prilosec®], [Zegerid®]), metoclopramide, erythromycin, and octreotide may be prescribed. Parenteral alimentation (feeding intravenously) may be used in severe cases.

Treatment for lung involvement depends on the severity of the condition. Calcium channel blockers, prostaglandins, antibiotics, high doses of corticosteroids (e.g., prednisone) may be prescribed. Epoprostenol (Flovan®) may be used to treat pulmonary hypertension. Severe lung disease may require a lung transplant.

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