Cyclophosphamide Fights Scleroderma
A drug that suppresses the immune system and reduces inflammation shows promise in treating a lung disease associated with the devastating connective-tissue disease scleroderma. Findings were presented at the American Thoracic Society International Conference in May.
A study found that a significantly higher proportion of patients taking cyclophosphamide, sometimes used as a treatment for cancer, showed improved lung function compared with those taking a placebo. They also had significant reductions in breathlessness and reported feeling more energetic and in better health. Known as the Scleroderma Lung Study, this is the first large, randomized, controlled trial to evaluate the drug’s effectiveness against scleroderma lung disease. Sponsored by the National Institutes of Health, the study was conducted at 13 centers throughout the U.S.
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Scleroderma is a progressive disease that leads to the hardening and tightening of the skin and connective tissues. About 80% of patients also have lung problems that lead to fibrosis. This scarring is caused by inflammation. The inflammation and scarring make breathing more difficult. More than 50% of scleroderma deaths are caused by lung-related problems.
About 150,000 Americans, mostly women, have scleroderma. The cause is unknown. Sixty percent to 70% of patients die within 10 years.
Researchers hope to study a combination of cyclophosphamide and another drug that will fight the effects of scarring.
