Venus Williams Battles Sjögren's Syndrome

Tennis ace Venus Williams pulled out of the US Open yesterday due to a battle with her toughest opponent yet, an energy-zapping autoimmunue disease that has left her so weak that she can barely raise her arms. “I enjoyed playing my first match here, and wish I could continue but right now I am unable to," Williams said in a statement, reported by the Associated Press. "I am thankful I finally have a diagnosis and am now focused on getting better and returning to the court soon."

The seven-time Grand Slam champion has been diagnosed with Sjögren's syndrome. Up to four million Americans are living with this chronic condition, which can spark extreme fatigue, joint pain, and increased risk for developing lymphoma, a form of cancer, or other serious complications. Like many people with Sjögren's, Williams suffered from symptoms for years without knowing what was wrong, but hopes that the treatments she’s now receiving will take effect within three to six months. Here’s a look at Sjögren's syndrome and what it might mean for the 31-year-old tennis ace.

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What is Sjögren's syndrome? Sjögren's syndrome (pronounced SHOW-grins) is one of the most prevalent autoimmune diseases. It’s a chronic condition in which the person’s white blood cells attack the body’s moisture-producing glands. The syndrome often strikes in tandem with other autoimmune conditions, including rheumatoid arthritis and lupus (a disease marked by chronic inflammation of organs and a butterfly rash across the nose).

What are the symptoms? The two classic hallmarks of Sjögren's syndrome are dry eyes that feel itchy or gritty, as if there’s sand in them, and a dry cottony mouth that makes it hard to swallow. Other symptoms include joint pain, swelling or stiffness, swollen salivary glands (particularly the ones behind the jaw and in front of the ears), dry nose or nosebleeds, heartburn, brain fog (trouble concentrating or remembering), tooth decay, abnormal liver function, numbness or tingling in the hands and feet, extreme fatigue, skin rashes, vaginal dryness, persistent bronchitis, pneumonia or lung disease, reports the Sjögren's Syndrome Foundation.

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How serious is Sjögren's syndrome? The disease can be mild to severe, but usually isn’t fatal. Patients need to be monitored for potential complications, including organ involvement, related autoimmune disease, dental problems (which are more common in people with Sjögren's syndrome due to dry mouth), and vision problems (resulting from dry eyes). Less common but worrisome complications include lymphoma (cancer of the lymph nodes), which is significantly more likely to occur in people with the syndrome), lung, liver or kidney disease, and nerve problems, such as peripheral neuropathy (numbness, tingling or burning sensations in the hands or feet). Many people with the condition lead normal, active lives.

Who is most likely to develop Sjögren's syndrome? 9 out of 10 people with this chronic autoimmune disease are women. The syndrome is most common after age 40, but can strike people of any age, including kids.

Is there a cure? Unfortunately, there’s no cure, but a variety of treatments can help relieve the symptoms, including OTC eye drops or prescription medications for dry mouth, including Evoxac (cevimeline), Salagen (pilocarpine hydrochloride) and Numoisyn for dry mouth and Restasis (cyclosporine ophthalmic emulsion) and Lacrisert (hydroxypropyl cellulose ophthalmic insert) for dry eye. For people who develop organ complications, immunosuppressant drugs may be prescribed.

How is the disease diagnosed? Sjögren's syndrome is often misdiagnosed or overlooked because it can mimic other conditions. Many patients, like Williams, have symptoms for several years before getting a diagnosis. There’s no one test for the syndrome, but an international panel of experts developed diagnostic criteria that include evaluating dryness symptoms, such as checking the eyes’ level of tear production and how much saliva the mouth produces. There are also several blood tests that doctors may use, including checking for ANAs (anti-nuclear antibodies). About 70 percent of people with the disease have a positive ANA test. To learn more about tests, click here.

What causes Sjögren's syndrome? The cause of this autoimmune disorder isn’t yet known, but certain genes raise the risk. In people who are genetically susceptible, some sort of triggering event may spark the disease, such as a bacterial or viral infection. In other words, while genes appear to load the gun, environment pulls the trigger. However, scientists haven’t yet discovered what the triggers are.

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