THURSDAY, Sept. 1 -- Venus Williams' surprising withdrawal from the U.S. Open on Wednesday has shifted the spotlight from the tennis star's daunting serve to her diagnosis with a little-known disease known asSjogren's syndrome.
Williams, 31, said the disease has diminished her energy level and caused joint pain, curtailing her ability to continue in the tournament.
More than 4 million Americans have the chronic autoimmune disease, which also causes dry mouth and dry eyes. Ninety percent of those with the disease are women.
Sjogren's syndrome "targets and destroys over time the exocrine glands responsible for tear production and saliva -- and is characterized by dryness of the mouth and eyes," said Dr. Michael Belmont, an associate professor of medicine in the division of rheumatology at NYU Langone Medical Center and medical director for Hospital for Joint Diseases. Less commonly, it can cause severe arthritis involving joint deformity and impaired function, he noted.
The publicity surrounding Williams' announcement is expected to increase awareness of the systemic disease, potentially leading to better diagnostic and treatment options.
"Sjogren's syndrome can occur as a primary disorder or secondary to other autoimmune diseases, principally rheumatoid arthritis or systemic lupus erythematosus [lupus]," Belmont said. Multiple organs, including the kidneys, lungs, gastrointestinal system, blood vessels and the central nervous system, can also be involved.
When Sjogren's progresses to a multi-organ disease, treatment becomes complex, said Dr. Victoria Shanmugam, a rheumatologist at Georgetown University Medical Center.
"Treatment for Sjogren's ranges from therapies to alleviate symptoms, such as topical therapies for dry eyes and dry mouth, to drugs that suppress the immune system, including hydroxychloroquine, methotrexate and steroids," said Shanmugam, who is not involved in Williams' care. "We also use medications that help fight fatigue and fibromyalgia symptoms."
Fibromyalgia is another chronic autoimmune disease.
Sjogren's, first identified in 1933, affects virtually every racial and ethnic group, according to the Sjogren's Syndrome Foundation. It is difficult to diagnose because symptoms mimic other diseases. It takes nearly seven years, on average, for a diagnosis.
How the disease will play out for Williams is unclear. Symptoms can remain mild, worsen or, occasionally, go into remission, Belmont said.
While the exact cause of Sjogren's syndrome is not known, Belmont said genetic factors appear to be involved. "An as-yet unidentified environmental factor likely plays a role, as observation finds that identical twins with the same DNA do not always share the illness," he said.
However, it is found more commonly in families that have members with other autoimmune illnesses, he said, noting this supports the genetic theory.
"Early diagnosis and proper treatment are important -- they may prevent serious complications and greatly improve a patient's quality of life," he said.
More information
To learn more about the disorder, visit the Sjogren's Syndrome Foundation.
Posted: September 2011
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