Venus Williams Diagnosed With Sjögren's Syndrome

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The human body seems to be under almost constant attack from the outside world, whether it is from harsh climate, from the sun's ultraviolet rays, or the bacteria and viruses that surround us. So, it seems especially unfair when the body attacks itself to cause illness. This is precisely what occurs in a group of illnesses referred to as autoimmune diseases. Autoimmune diseases can affect almost anyone, and the latest patient in the spotlight is Venus Williams, who withdrew from the U.S. Tennis Open because of Sjögren's syndrome.

"I'm really disappointed to have to withdraw from this year's U.S. Open," Williams said in a statement. "I have recently been diagnosed with Sjögren's syndrome, an autoimmune disease which is an ongoing medical condition that affects my energy level and causes fatigue and joint pain...I enjoyed playing my first match here and wish I could continue, but right now I am unable to. I am thankful I finally have a diagnosis and am now focused on getting better and returning to the court soon." – Venus Williams in USA Today.

Imagine what happens when lymphocytes, a type of white blood cell, decide that organs in the body are alien and don't belong. They mount an attack and invade and infiltrate that organ, causing it to fail. That is autoimmune disease in a nutshell. In Sjögren's syndrome, the lymphocytes attack glands whose secretions pass through a duct directly to the outside of the body. The most common complaints are dry eyes and dry mouth, since the glands that make tears and saliva are affected. (It's important to remember that most people with dry eyes and dry mouth have it because of other, non-serious causes.) Sjögren's syndrome would less serous if those were the only issues, but the syndrome can also involve the lungs, kidney, liver, and skin. Moreover, it can rarely be associated with a form of cancer of the lymph glands called lymphoma.

There are plenty of theories as to what causes the autoimmune attack to start, and there may be an association with rheumatoid arthritis, systemic lupus erythematosus, and scleroderma. The diagnosis of Sjögren's syndrome is often challenging because patients often present with nonspecific complaints such as fatigue, joint ache and swelling, abdominal pain, and diarrhea (as well as the classic dry eyes and dry mouth).

It's also a tough diagnosis because there isn't total agreement as to criteria for the diagnosis. There can be abnormal blood tests, including autoantibodies (rheumatoid factor or RF, and antinuclear antibody or ANA), and tests looking for inflammation such as erythrocyte sedimentation rate or ESR. There are antibodies in the blood that can be found in many, but not all patients with the Sjögren's syndrome. These antibodies are referred to as Sjögren's syndrome antibodies A and B (also known as SSA or Ro antibody and SSB or La antibody). Biopsies of salivary glands can be done to help confirm that lymphocytes have invaded the gland. There are consensus guidelines for making the diagnosis, but mostly it comes down to the doctor having a high index of suspicion and pursuing it.

If only the eyes and mouth are involved, treatment is aimed to control symptoms, but aggressive medications may be needed if major organs like the lung, liver, and kidney are involved. Again, another concern is that patients can (though uncommonly) develop non-Hodgkin's lymphoma cancer. Most patients, however, do well and their prognosis is based on whether other autoimmune diseases like lupus and rheumatoid arthritis are also involved.

When an athlete develops an uncommon disease, the only upside is the publicity and awareness that it brings. Hopefully, Ms. Williams will continue to perform as an elite athlete and perhaps some of her fans may see in her symptoms their own situation and be able to work through it.


USA Today

Last Editorial Review: 8/31/2011 8:04:38 PM