Tennis star Venus Williams withdrew from the US Open yesterday, citing difficulties with Sjögren’s syndrome, an autoimmune disease that affects about 4 million Americans. Like other autoimmune diseases, the immune cells of people with Sjögren’s attack the body, hitting the tear- and saliva-producing exocrine glands in particular. Thus, common symptoma of the syndrome are dry eyes and mouths — but it can also lead to joint paint and fatigue if the immune cells target other areas.
“The fatigue is hard to explain unless you have it,” Williams told the New York Times. “Some mornings I feel really sick, like when you don’t get a lot of sleep or you have a flu or cold. I always have some level of tiredness. And the more I tried to push through it, the tougher it got.”
But what can she do about it? There are few treatments available for the syndrome, which disproportionately affects those from Venus (nine out of ten sufferers are women). Many treatments used for lupus, a far more severe autoimmune disorder, are being tested in people with Sjögren’s. These include hydroxychloroquine, an antimalarial drug that tones down inflammatory and immune responses, which the Paris Hospital and the French drugmaker Sanofi are testing in a 120-person, phase 3 trial for Sjögren’s. Another immune inhibitor, belimumab, which is often used to treat lupus, has been registered for two phase 2 trials. And rituximab, an antibody that kills immune B-cells, is being tested for Sjögren’s by the University Hospital in Brest in France in collaboration with the country’s Ministry of Health.
A bolder trial, currently recruiting, plans to use stem cell therapy to modulate the disease. The researchers from Nanjing University Medical School in China published a paper in 2009 suggesting that dysfunctional stem cells in the bone marrow cause autoimmunity, and found that this therapy brought four lupus patients into disease remission. Bone marrow stem cells differentiate into immune cells; if they are defective, they could disable the immune system, according to their work. But if the marrow could be replaced with new stem cells, perhaps the disease could be improved — or cured.
Williams herself is most likely taking some of the lupus pills, which are often prescribed off-label for Sjögren’s. It’s a backhanded way to combat the disease, but hopefully one that will give her an advantage.
Image: by Brett Weinstein, on Wikimedia Commons under Creative Commons license
