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April 2002
Vol. 5, No. 4, p 64.
diseases and disorders
Mary Anne Dunkin

Sjogren's Syndrome

figureIf you are a woman and sexual intercourse has been painful for you lately, would you share that information with your dentist or eye doctor? If your eyes felt gritty or your last dental checkup revealed cavities, would you even think to mention it to your gynecologist? Most women would not. And that, probably, is one of the main reasons that Sjogren’s syndrome, a common medical condition in women, often goes undiagnosed and untreated. Named for Henrick Sjogren, the Swedish physician who first diagnosed the condition in the 1930s, Sjogren’s syndrome affects an estimated 1–4 million Americans, approximately 90% of whom are women. Because the key symptoms of the condition—dryness of tissues, including those of the eyes, mouth, and vagina—are often not connected, getting a more precise handle on the number of cases can be difficult. Although the symptoms of Sjogren’s syndrome are diverse and seemingly unrelated, they have a common cause: an immune system that mistakenly attacks and destroys the body’s moisture-producing glands.

For many women, Sjogren’s syndrome is little more than a nuisance: A parched mouth gives them insatiable thirst, gritty eyes make reading difficult, and a dry, painful vagina interferes with sexual pleasure. But for some, the problem goes further. A deficiency of saliva can lead to serious tooth decay and oral infections, dry skin can crack and become infected, and a lack of eye lubrication can lead to corneal ulcers and even loss of vision. In a small percentage of women, the problem gets even worse, affecting the lungs, kidneys, and digestive and nervous systems. And, in about 5%, it leads to potentially fatal lymphoma.

Unknown cause and cure
Sjogren’s syndrome is classified as either primary or secondary. Primary Sjogren’s is separate from any other disease process. Secondary Sjogren’s occurs in the presence of another connective tissue disease, such as lupus, scleroderma, or rheumatoid arthritis. The average age of occurrence is late 40s.

Although the cause of Sjogren’s syndrome is unknown, genes and viral infections are believed to play a role in its development. As for why women are so much more likely to be affected, scientists must return to the age-old question as to why most autoimmune diseases affect women disproportionately. Suspected answers involve female hormones and the fact that women’s immune systems are naturally more active than men’s.

Making the diagnosis
Because any number of factors—including menopause and many prescription and over-the-counter medications—can cause tissue dryness, symptoms alone cannot confirm a diagnosis of Sjogren’s syndrome. However, several laboratory tests aid in diagnosis. They include

  • Schirmer’s test to measure the function of the lacrimal gland;
  • slit lamp examination to determine the extent of eye dryness and inflammation;
  • salivary gland biopsy; and
  • immunologic blood tests to check for the presence and number of certain antibodies that may indicate Sjogren’s syndrome, including immunoglobulins, gamma globulins, rheumatoid factor, antinuclear antibodies, and Sjogren’s antibodies (SS-A and SS-B).

Restoring moisture
figureAlthough there is no cure for Sjogren’s, scientists are making headway. For example, gene therapy studies suggest that doctors someday may be able to insert compounds into the salivary glands that will control inflammation and prevent their destruction. Work on developing an artificial salivary gland is also in progress. Until such research comes to fruition, the treatment of Sjogren’s syndrome is targeted at easing symptoms. For some women, keeping a water bottle or saliva substitute handy for dry mouth, or using over-the-counter products such as artificial tears (eyedrops) and vaginal lubricants to ease eye and vaginal dryness, is sufficient. More serious dryness requires stronger medication. Two prescription drugs, pilocarpine hydrochloride (Salagen) and cevimeline hydrochloride (Evoxac), are approved by the FDA for treating the dry mouth of Sjogren’s syndrome. Rather than masking dryness, these drugs increase salivary flow. There is evidence that they may also ease dry eyes.

Glucocorticoid medications such as prednisone are used to control organ inflammation and prevent some of the more serious complications of the disease. Regardless of its severity, self-care is important, and people with Sjogren’s syndrome who have persistent enlargement of the salivary glands or swollen lymph nodes—possible symptoms of lymphoma—should see a doctor regularly.

Further information
National Institute of Arthritis and Musculoskeletal and Skin Diseases; www.niams.nih.gov/hi/topics/sjogrens/index.htm.
Sjogren’s Syndrome Foundation, Inc.; www.sjogrens.org.

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