Treatment for Lung Complications

There are 2 main types of scleroderma-related lung complications:

• Pulmonary fibrosis
• Pulmonary hypertension

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, in both intravenous and oral forms, blocks some of the destructive actions of scleroderma in the lung. Intravenous (I.V.) cyclophosphamide can be life saving for patients with pneumonia 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.

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Use of this agent may improve survival in patients who show early signs of lung deterioration, notably inflammation in the small lung airways (called alveolitis). The drug is not warranted 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 two years.

Pulmonary Hypertension

Several types of drugs are used to treat pulmonary hypertension. Oral anticoagulants, 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.