Funding outcome

Systemic sclerosis (SSc) is a serious, debilitating disease that causes the skin in the arms and legs to become thick, tight, and scarred. In about 1/3rd of patients, this hardening of the skin also extends into the heart and lungs, which is extremely dangerous. There is no cure for SSc and people with the disease live an average of 20 years shorter lives than healthy adults - especially devastating given that the average age of diagnosis is 30 - 50 years old. Our lab is interested in understanding how SSc causes disease in women, since women are 10 times more likely to develop SSc than men and, unlike men, it is virtually impossible to predict how the disease will affect women. Unfortunately, doctors are still unsure why the disease affects women differently. Without understanding what causes the disease or being able to predict how the disease will progress, it is extremely hard for doctors to treat their women patients. Our research focuses on a special sugar molecule called polysialic acid (polySia), which is normally found on the surface of immune cells that protect our bodies. This sugar is normally absent from skin. However, we discovered that it shows up in the skin of patients with SSc and that women who are sicker have more polySia. We also discovered that the skin cells in women with SSc, but not men, secrete the sugar into the bloodstream where it can be detected with a simple blood test. We now want to know if these changes in polySia can predict which women will develop more serious illness. If polySia turns out to be an early warning sign, doctors could use it to find high-risk patients sooner and give them better, more personalized care. This research could lead to new ways to help women with SSc live longer, healthier lives by improving how we predict and treat the disease.