Scleroderma is a rare disease that commonly involves the lungs in a variety of ways. These include interstitial lung disease, pulmonary vascular disease and bronchiolitis. Pulmonary hypertension is the leading cause of mortality in scleroderma. The treatment of scleroderma, generally, and its associated lung diseases, specifically, have not been well defined by controlled trials. However, the results of uncontrolled studies and an understanding of the pathological basis of scleroderma allow a rational choice of therapeutic options.
With our current limited knowledge, a reasonable approach is to treat progressive interstitial lung disease with immunosuppressive drugs such as cyclophosphamide and prednisone. Pulmonary vascular disease has a poor prognosis and therapy is currently limited, although new treatment strategies are on the horizon. Most promising is the recent development of locally administered therapies. These may overcome the frequent and excessive adverse effects that limit the use of potent systemically administered drugs.