Systemic sclerosis (also known as scleroderma) is an autoimmune disease that results in damage to and hardening of the skin, blood vessels, lungs, heart, kidneys and gastrointestinal tract. One group of researchers has found a potential clue to the development of the condition, and another group has had success in treating a leading cause of death among people with scleroderma.

The primary cause of systemic sclerosis is not known, but an Italian research team identified antibodies against platelet-derived growth factor receptors (PDGFR) in people with scleroderma (New England Journal of Medicine, June 22, 2006). These antibodies trigger a chemical cascade that results in increased collagen production, which is the hallmark of scleroderma and causes organ damage. Their experiments strongly indicate that these PDGFR antibodies have a role in causing scleroderma; with that knowledge, new therapies that block the action of the antibodies can now be developed.

Interstitial lung disease is a consequence of scleroderma and is a leading cause of death among people with scleroderma. The Scleroderma Lung Study Research Group, a large multicenter group of scientists, released results of a well-controlled clinical trial of oral cyclophosphamide (Cytoxan) on lung function in people with scleroderma interstitial lung disease (New England Journal of Medicine June 22, 2006). After one year of treatment, people in the trial had a significant but modest improvement in lung function, less shortness of breath, reduced skin thickening, and a higher health-related quality of life. Treatment of scleroderma has been challenging, and this study provides evidence for the treatment of scleroderma lung disease, one of the most severe complications of the disease.