番茄社区

When former No. 1 women's tennis star Venus Williams withdrew from the U.S. Open this week, she brought immediate attention to the little-known autoimmune disease Sjögren's syndrome, thought to afflict some 0.5% of adults.

The disease is characterized by destruction of the exocrine glands, leading to excessive dryness of the mouth and eyes.

But Sjögren's syndrome also is associated with symptoms such as debilitating fatigue and musculoskeletal problems that could seriously interfere with athletic performance.

"It's my suspicion that it may have been the systemic manifestations like fatigue and joint pain that have been troubling her and that could be difficult for a person trying to function at that very physically demanding level," Robert Spiera, MD, director of the vasculitis and scleroderma program at the Hospital for Special Surgery in New York City, told 番茄社区. Spiera has not been involved in Williams' treatment.

Earlier in the week Williams, 31, won a match in the tournament, held at the Billie Jean King National Tennis Center in Flushing, N.Y., but when she left the venue, after withdrawing on Wednesday, she said in a statement, "I enjoyed playing my first match here and wish I could continue, but right now I am unable to," according to the

Steven Taylor, who heads the Sjögren's Syndrome Foundation, said in a statement, "On behalf of the four million Americans with Sjögren's, we applaud Venus for publicly stepping forward and shedding light on this serious autoimmune disease."

Diagnosing the disease can be challenging, and can involve salivary flow testing, salivary gland biopsy, and blood tests for autoantibodies such as rheumatoid factor, antinuclear antibodies, SSA and SSB antibodies.

Williams, who has been sidelined in recent months because of what was thought to be a viral illness, said she was "thankful to finally have a diagnosis." In fact, according to Taylor, the average time to diagnosis is 6.5 years after symptom onset.

The condition affects women far more than men, as is the case with most autoimmune diseases, and generally begins when patients are in their 40s.

"When patients present with complaints such as fatigue, clinicians need to be proactive and think beyond the common diagnoses like depression and thyroid disorders and ask about dry eyes and mouth," Spiera said.

Some similarities exist between Sjögren's syndrome and other autoimmune disorders such as rheumatoid arthritis and lupus, including articular, renal, and neurologic involvement.

For many patients, symptoms can be managed with products such as eye lubricants.

But for those with more severe manifestations, systemic medications such as anti-inflammatories, antimalarial drugs, and immunosuppressive agents may be needed.

Williams apparently is taking an oral medication that could take several months for its full effects to be seen, according to another in the Times.

B cells are thought to play a crucial part in the development of Sjögren's syndrome, accumulating and becoming hyperactive in the exocrine glands and heightening immune antigenic responses.

For reasons that are not yet clear but which may involve B-cell proliferation and mutagenicity, in some patients the immune hyperactivity can evolve into a lymphoproliferative malignancy.

"Patients with Sjögren's have a risk of lymphoma that is as much as 40-fold higher than the general population," Spiera said.

Certain features of the disease seem to be associated with the development of lymphoma, such as bulky lymph or parotid gland enlargement, skin vasculitis, and low blood levels of complement.

"But the vast majority of patients with Sjögren's syndrome never develop lymphoma," Spiera said.

"Most patients with the disorder live very productive lives. Sjögren's does affect their quality of life, but there are strategies to help deal with their problems," he added.

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