Excess collagen is deposited in the skin and organs, causing the skin to thicken and harden and sometimes affecting the function of internal organs. It affects women three to five times more often than it affects men. Approximately 100,000 to 165,00 people in the U.S. have the disease. People with lung damage caused by scleroderma generally have shortness of breath. Because of aggressive medical treatment, more people are living longer with the disease and about 40 percent of patients develop severe lung problems.
Lung disease is now the leading cause of death for scleroderma patients.
Patients in the study will be chosen randomly to receive either the drug or the placebo. The drug is currently approved by the U.S. Food and Drug Administration for use in malignant lymphoma, multiple myeloma, leukemia, neuroblastoma and other diseases, but not for scleroderma. The two-year study will enroll 163 participants nationwide, with 16 enrolled in Pittsburgh.