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Scleroderma

Description

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

Alternative Names

Systemic sclerosis

Treatment for Lung Complications

Pulmonary Fibrosis

Cyclophosphamide. Cyclophosphamide (Cytoxan), an immunosuppressive agent, may be effective for preventing lung deterioration and is the important agent for treating pulmonary fibrosis. Cyclophosphamide, available in forms that are intravenous and taken by mouth, blocks some of the destructive actions of scleroderma in the lung. Intravenous cyclophosphamide can be life-saving for patients with pneumonia resulting from interstitial lung disease. Side effects include hair loss, infection, and bleeding into the urinary tract. To date, no other immunosuppressive agents have proven to have any significant benefits.

Use of this drug may improve survival in patients who show early signs of lung deterioration, notably inflammation in the small lung airways (alveolitis). The drug is not recommended for patents with existing stable pulmonary fibrosis and no signs of inflammation. In one study, patients with early signs of lung inflammation were given a course of intravenous pulses of the corticosteroid methylprednisolone (MP) and cyclophosphamide. Nearly all patients experienced improvement or stabilization during the first year, although the disease had progressed in two-thirds of them by the end of 2 years.

Pulmonary Hypertension

Several types of drugs are used to treat pulmonary hypertension. Anticoagulants taken by mouth, such as warfarin (Coumadin), are a standard treatment used to prevent blood clot formation. Diuretic treatment and supplemental oxygen are recommended for patients with fluid retention and low blood oxygen, respectively.

Vasodilators help to open blood vessels and relieve pressure in arteries in the lungs. Vasodilators used to treat pulmonary hypertension fall into several different drug classes:

Calcium Channel Blockers (CCBs). Some patients with pulmonary hypertension benefit from these drugs. They help relax blood vessels in the heart and lungs, and increase the supply of oxygen. However, calcium channel blockers are only appropriate for patients who meet certain diagnostic criteria, including absence of right-sided heart failure.

Prostacyclins (Prostaglandins). Prostacyclins, which open blood vessels, are now the primary agents for treating pulmonary hypertension.

• Iloprost (Ventavis) is available in inhaled and intravenous forms. Studies suggest that the inhaled form improves exercise capacity and survival in some patients with pulmonary hypertension. In addition, infusions of iloprost remain effective over long periods (up to 3 years) of use.
• Treprostinil (Remodulin) is similar to epoprostenol but is more stable. It can also be administered using a portable pump that delivers the drug under the skin. This is less expensive, cumbersome, and invasive than the delivery methods for epoprostenol.
• Epoprostenol (Flolan), which is administered intravenously, has improved exercise capacity and symptoms in both the short and long term in a number of patients. In some patients, survival is increased significantly. However, not all patients respond to this agent. The implanted catheter needed to deliver the agent can also cause serious complications.

Endothelin Receptor Antagonists. Bosentan (Tracleer) was the first drug taken by mouth that was approved for pulmonary hypertension. Bosentan controls endothelin, a powerful molecule that causes blood vessels to narrow. Studies have reported improved exercise capacity in patients with pulmonary hypertension.

PDE5 Inhibitors . Sildenafil (Revatio) was approved in 2005 as the first pill for patients with early-stage pulmonary hypertension. Sildenafil is the same agent contained in the erectile dysfunction drug Viagra. However, Revatio is prescribed at a lower dosage than Viagra, and is a different color and shape than Viagra pills.

Other Treatments . Lung transplantation may offer hope for people with advanced pulmonary hypertension that does not respond to conservative measures.

• 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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