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Promising New Research May Have Impact on Scleroderma Population

Key Peptide that Blocks Skin, Lung Fibrosis Could Improve Mortality Rates


Carol Feghali-Bostwick photoDANVERS, MASS. (May 30, 2012) – A member of the Scleroderma Foundation’s Board of Directors has led a team of researchers who have identified a peptide that can block fibrosis of the skin and lungs, according to a report published today. “Lung fibrosis currently is the number one cause of death in patients with scleroderma,” said the report’s senior author Carol A. Feghali-Bostwick, Ph.D. “Identifying a way to stop this process from happening could have enormous impact on mortality and quality of life.” Dr. Feghali-Bostwick is vice chair of the Scleroderma Foundation’s Board of Directors and chair of the Foundation’s Research Committee.

In the research reported today in Science Translational Medicine, investigators were looking into agents that promote fibrosis when, by happenstance, they discovered the peptide, a stretch of amino acids representing a small piece of a protein that stopped fibrosis in mice.

Tissue fibrosis contributes to 45 percent of all deaths in developed countries, according to Dr. Feghali-Bostwick. To determine if the peptide, called E4, is relevant for human disease, investigators tested it in human skin samples maintained in the laboratory. The results indicated that the peptide was successful at reducing fibrosis in human skin. This suggests that the peptide has a high likelihood of being effective in humans with fibrosis. “Identification of a molecule that stops fibrosis in both skin and lung brings us closer to a possible treatment for fibrosis. Currently, the only therapy for lung fibrosis is transplantation,” she said.

These findings emphasize the importance of supporting research on scleroderma and related diseases. “This should be a call-to-action as to why more dollars for scleroderma research is essential,” said Joseph P. Camerino, Ph.D., chair of the Foundation’s Board of Directors. “Many investigators who have been previously funded by the Foundation have become leaders in their field. This indicates the importance of investing in research for scleroderma. It ensures that the research will continue so we can find the cause and a cure for this devastating disease.” Early in her career, the Scleroderma Foundation supported Dr. Feghali-Bostwick. In 1999, she received the Foundation’s New Investigator Grant to study twins with scleroderma.

“This new data highlights the rapid progress being made in scleroderma research,” said John Varga, M.D., director of the Scleroderma Program and professor of medicine at Northwestern University’s Feinberg School of Medicine, and also chair of the Scleroderma Foundation’s Medical Advisory Board. “These are exciting findings, but we need to remember that the new compound is not the cure. Rather, it is a piece to the puzzle that will eventually lead us to find the cure.”

Dr. Feghali-Bostwick is an associate professor, Division of Pulmonary, Allergy and Critical Care Medicine, and co-director of the Scleroderma Center at University of Pittsburgh School of Medicine.

Other co-authors of the study include Yukie Yamaguchi, M.D., Ph.D., Takahisa Takihara, M.D., Roger A. Chambers, B.S., and Kristen L. Veraldi, M.D., Ph.D., of the Division of Pulmonary, Allergy and Critical Care Medicine, the Department of Pathology, and the Scleroderma Center, University of Pittsburgh School of Medicine; and Adriana T. Larregina, M.D., Ph.D., of the Departments of Dermatology and Immunology, University of Pittsburgh School of Medicine, and the McGowan Institute for Regenerative Medicine.

The University of Pittsburgh research project was funded by National Institutes of Health grant AR050840.

For more information, visit the Science Translational Medicine website at

Editor’s Note: To arrange an interview with Dr. Feghali-Bostwick, please contact Christina Relacion at (800) 722-4673, Ext. 243, or email


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