New York, NY—October 28, 2016
The prognosis for patients diagnosed with scleroderma - an autoimmune disease characterized by fibrosis of the skin - is not typically a rosy one. With limited treatment options available, those suffering from the disorder can face disabling hardening and tightening of their skin. Scleroderma can also affect the blood vessels, lungs and other internal organs.
New and ongoing research at Hospital for Special Surgery in New York City has identified a possible mechanism behind the fibrosis that occurs in scleroderma – a mechanism that may one day lead to a treatment for the disease.
Published in the Journal of Clinical Investigation on October 10, the study reports that in laboratory research, a population of stem cells called 'adipose-derived stromal cells' (ADSCs) is reduced in number in the layer of fat sitting under the skin. It appears that loss of these ADSCs may contribute to the skin fibrosis characteristic of scleroderma.
Moreover, the study authors found that the survival of those ADSCs that do remain beneath the skin in scleroderma are dependent on immune cells called 'dendritic cells'. Dendritic cells release a compound called lymphotoxin B that promotes ADSC survival; when antibodies that stimulate the lymphotoxin B receptor were administered with ADSCs to replenish the lost ADSCs, ADSC survival was found to be increased, suggesting a means for reversing the fibrosis of the skin.
"Injecting ADSCs is being tried in scleroderma; the possibility of stimulating the lymphotoxin B pathway to increase the survival of these stem cells is very exciting," says lead study author Theresa T. Lu, MD, PhD. "By uncovering these mechanisms and targeting them with treatments, perhaps one day we can better treat the disease."
Dr. Lu also feels this strategy could be used to target stem-cells from other tissue sources in order to treat rheumatological and other conditions - such as lupus and rheumatoid arthritis - and also to facilitate bone and cartilage repair.
In the coming years, Dr. Lu and her colleagues hope to test the applicability of their work in human cells, which could provide scleroderma patients with a welcome treatment option if proven safe and effective. "Improving ADSC therapy would be a major benefit to the field of rheumatology and to patients suffering from scleroderma," she says.
Other HSS authors include first author Jennifer Chia, Tong Zhu, Susan Chyou, Dragos Dasoveanu, and Camila Carballo, and HSS faculty members Drs. Jessica Gordon and Robert Spiera of the HSS Scleroderma and Vasculitis Center and Dr. Scott Rodeo of the Sports Medicine and Shoulder Service.
About HSS | Hospital for Special Surgery
HSS is the world’s leading academic medical center focused on musculoskeletal health. At its core is Hospital for Special Surgery, nationally ranked No. 1 in orthopedics (for the ninth consecutive year) and No. 3 in rheumatology by U.S.News & World Report (2018-2019). Founded in 1863, the Hospital has one of the lowest infection rates in the country and was the first in New York State to receive Magnet Recognition for Excellence in Nursing Service from the American Nurses Credentialing Center four consecutive times. The global standard total knee replacement was developed at HSS in 1969. An affiliate of Weill Cornell Medical College, HSS has a main campus in New York City and facilities in New Jersey, Connecticut and in the Long Island and Westchester County regions of New York State. In 2017 HSS provided care to 135,000 patients and performed more than 32,000 surgical procedures. People from all 50 U.S. states and 80 countries travelled to receive care at HSS. In addition to patient care, HSS leads the field in research, innovation and education. The HSS Research Institute comprises 20 laboratories and 300 staff members focused on leading the advancement of musculoskeletal health through prevention of degeneration, tissue repair and tissue regeneration. The HSS Global Innovation Institute was formed in 2016 to realize the potential of new drugs, therapeutics and devices. The culture of innovation is accelerating at HSS as 130 new idea submissions were made to the Global Innovation Institute in 2017 (almost 3x the submissions in 2015). The HSS Education Institute is the world’s leading provider of education on the topic on musculoskeletal health, with its online learning platform offering more than 600 courses to more than 21,000 medical professional members worldwide. Through HSS Global Ventures, the institution is collaborating with medical centers and other organizations to advance the quality and value of musculoskeletal care and to make world-class HSS care more widely accessible nationally and internationally.