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Venus Williams and Sjogren’s Syndrome: Nothing to Drool Over

Shedding no tears.

It was recently reported that Venus Williams was diagnosed with Sjogren's syndrome, an illness that led to her recent and last-minute withdrawal from the U.S. Open. Many have never heard of this disease, and thus a common question is: Will it happen to me?

According to some sources, as many as 4 million Americans suffer from this autoimmune disease. The disease may occur in a primary form, without features of other rheumatologic disorders, or in a secondary form, associated with illnesses such as systemic lupus erythematosus (SLE), rheumatoid arthritis, or scleroderma. Primary Sjogren's syndrome is found predominantly in women, who are of the approximate age of Ms. Williams at the time of disease onset. Given the overlap, it is sometimes difficult to determine whether a particular clinical manifestation is solely a consequence of Sjogren's syndrome, or due to a concurrent disorder.

This chronic inflammatory disorder is characterized by white blood cell infiltration of the organs that produce secretions, including the tear glands and the salivary glands. Patients with Sjogren's syndrome most often describe dryness of the eyes and mouth, these being the most frequent presenting symptoms. But these symptoms can have an insidious onset, and some patients might be diagnosed many years later, only after presenting to the dentist with horrible tooth decay (due to the absence of the detergent action of the enzymes in saliva), or to the ophthalmologist with corneal abrasions (due to the absence of the lubricating effect of tears). Vaginal dryness can be both physically and psychologically distressing, and lead a more shy patient to delay a visit to the doctor.

However, so-called extraglandular features may bring patients to a physician first, as joint pain, muscle pain, skin rash, shortness of breath, or generalized fatigue are often experienced by patients suffering from Sjogren's syndrome. Neuropsychiatric manifestations may be the only manifestations initially; and in fact I once had a patient who had first been admitted to the psychiatric ward for a presumed psychotic episode, finally transferred to the medicine team after a few days of lack of responsiveness to a variety of anti-psychotic drugs.

Classic clinical features of Sjogren's syndrome may be seen in viral infections, including hepatitis C, HIV, and human T cell lymphotropic virus (HTLV). Interestingly, it was reported that Ms. Williams experienced a viral illness that prevented her from playing in a warm-up tournament prior to the U.S. Open. One would imagine that her physicians would have performed a complete work-up when she presented with her complaints.

Treatment of Sjogren's syndrome is based on a given patient's symptoms:
• Dry eyes are treated with artificial tears.
• Dry mouth is treated with sugar-free lemon drops and artificial saliva.
• Vaginal lubricants are helpful with vaginal dryness.
• Medications such as Evoxac can stimulate the salivary glands to produce more saliva.
• Immunosuppressant drugs are beneficial when treating kidney or nerve involvement.

Venus Williams has been a tremendous asset to tennis. It will be nice to see her back on the court in the very near future.