Jefferson researchers to investigate mechanisms involved in nephrogenic systemic fibrosis

Researchers at the Jefferson Institute of Molecular Medicine were awarded almost $384,000 by the National Institutes of Health to investigate the mechanisms involved in the development of a serious and sometimes fatal disease, known as Nephrogenic Systemic Fibrosis, a rare skin disorder linked to Gadolinium-based contrast agents used in magnetic resonance (MRI and MRA) studies.

The team, led by Sergio A. Jimenez, M.D., Professor in the Department of Dermatology and Cutaneous Biology at Thomas Jefferson University, Co-Director of the Jefferson Institute of Molecular Medicine, and Director of the Jefferson Scleroderma Center, aim to gain a better understanding of the molecular mechanisms behind the initiation of the disorder in order to develop possible new treatments.

They will investigate the role of specialized cells known as macrophages and their cell surface receptors known as Toll-like receptors in Gadolinium-based contrast agent induced skin fibrosis.

"Using mouse models, we can specifically induce the disease to better examine the pathways responsible for the development of the severe and progressive fibrosis of skin and internal organs, and hopefully find weaknesses to exploit," said Dr. Jimenez. "The results could help us identify promising molecular targets for the development of new therapies for this as well as other fibrotic disorders, including Scleroderma."

Nephrogenic Systemic Fibrosis is a rare and serious disorder that involves progressive fibrosis of skin, joints, and numerous internal organs. It is induced by exposure of patients with renal failure to gadolinium-based contrast agents employed for magnetic resonance studies.