Understanding Systemic Sclerosis: New Research Finds Piece of the Puzzle

Systemic sclerosis is a chronic and devastating illness. There are no successful treatments, and mortality rates can reach to over 50%. New research by a team at Hospital for Special Surgeries in New York City, however, may provide a hope for those suffering from systemic sclerosis. Keep reading to learn more, or follow the original story here.
Systemic sclerosis, or scleroderma, is an autoimmune disorder that primarily affects the body’s connective tissue. It causes inflammation, and fibrosis, and can result in severe damage to the skin. Current treatment for systemic sclerosis relies on treating symptoms. This has been the case for decades, and even then treatment has inconsistent results. The underlying causes and workings of systemic sclerosis remain poorly understood. To learn more about it, click here.

Recent research published in Science Translational Medicine, however, may contain a breakthrough.

There is also hope that this new understanding will lead to effective treatment. Dr. Franck Barrat, Michael Bloomberg chair and senior scientist at Hospital for Special Surgeries, led the research team. The team’s work points to plasmacytoid dendritic cells as a key player in systemic sclerosis.

Under normal conditions, plasmacytoid dendritic cells (pDCs) produce interferon which is used to help battle infections.

Researchers discovered that in patients with systemic sclerosis pDCs are overactive.

The cells become frequently enabled and infiltrate the skin. When pDCs behave in this way the body responds with inflammation, and fibrosis occurs. Researchers also pinpointed a receptor called TLR8 as the cause of the overactive pDCs.

Other rheumatoid conditions also exhibit strange behavior by pDCs. Lupus, for example, shares the over active pDC quality with systemic sclerosis. Dr. Barrat explains that the team’s findings not only point to a similarity in cause, but also establish the role of pDCs in beginning and continuing the fibrosis process in the skin.

“This is a very interesting finding as it opens new ways to tackle this condition,” says Dr. Barrat.

Dr. Barrat’s research also contains an interesting finding in animal models. He discovered that if pDCs were depleted that systemic sclerosis would not form, and that existing fibrosis could be turned back. Dr. Barrat is careful to acknowledge that systemic sclerosis is simpler in animal forms, and that his research may not yet be ready to create a cure. He is hopeful, however, that his research will lead to more and more understanding and future developments.

Techniques to dispose of pDCs are currently being investigated by pharmaceutical companies in relation to other diseases. Treatments are years from becoming available, but Dr. Barat expresses an ambition that perhaps having better knowledge of pDC functions will allow some of those treatments to also be used in therapies for systemic sclerosis.

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