Scleroderma is an autoimmune
disease characterized by fibrosis of the skin. The prognosis for patients diagnosed with scleroderma 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
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.
‘Loss of adipose-derived stromal cells (ADSCs) may contribute to the skin fibrosis characteristic of scleroderma.’
Published in the Journal of Clinical Investigation
, 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
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. "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.