Progressive systemic sclerosis: Part 1--The disease and medical management.

The incidence of scleroderma is approximately 700,000 people in the United States. Pathologic changes occur in a variety of body systems and can alter lifestyle, especially for people with diffuse scleroderma. Information from clinical practice and research concerning the nature, incidence, pathophysiology, complications, and medical management is discussed. Although there is no known cure for scleroderma, life expectancy has improved as management of renal hypertensive crisis and renal failure has improved.