As Venus Williams Bows Out of U.S. Open, Little-Known
Disease Takes Center Court09/01/11
THURSDAY, Sept. 1 (HealthDay News) -- 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 as Sjogren's syndrome.
Williams, 31, said the disease has diminished her energy level
and caused joint pain, curtailing her ability to continue in the
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
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 hydroxychloroque, methotrexate and steroids," said Shanmugam, who is not involved in Williams' care. "We also use medications that help fight fatigue and fibromyalgia symptoms."
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
How the disease will play out for Williams is unclear. Symptoms
can remain mild, worsen or, occasionally, go into remission,
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
"Early diagnosis and proper treatment are important -- they may prevent serious complications and greatly improve a patient's quality of life," he said.
To learn more about the disorder, visit the
Sjogren's Syndrome Foundation.
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