Sjogren's Syndrome

By Marilyn Haddrill; review and additional contributions by Charles Slonim, MD

Sjogren's syndrome (pronounced SHOW-grins, and technically spelled Sjögren's) is an autoimmune disease that attacks and destroys glands responsible for keeping the eyes, mouth and other parts of the body moist and lubricated. For this reason, dry eyes are a common symptom.

Older women beyond menopause are more likely to develop Sjogren's syndrome (SS), for unknown reasons. In fact, about 2 percent of all women older than 60 are thought to have some form of the disease, according to Kelley's Textbook of Rheumatology, 7th edition.

The Sjogren's Syndrome Foundation estimates that as many as 4 million U.S. residents have the disease, and about 90 percent are women.

Causes of Sjogren's Syndrome

The condition occurs alone in its primary form. In its secondary form, Sjogren's syndrome develops in association with other autoimmune disorders such as systemic lupus erythematosus (SLE), thyroiditis or rheumatoid arthritis. These conditions cause inflammatory responses in different parts of the body.

Why abnormal immune responses destroy a body's own glands is not clearly understood. Ordinarily, our body's immune system recognizes our own body parts as "friendly," but becomes activated to fight and destroy "foreign" substances such as poisons, foreign bodies or harmful organisms, such as viruses.

The kinds of abnormal immune responses that inexplicably destroy parts of our own body may be inherited, or they may relate to viral or bacterial infections.

Several recent studies have shown that a significant number of people who have celiac disease, an abnormal immune response to gluten in wheat, also have Sjogren's syndrome, according to the June 2008 issue of Gastroenterology Clinics.

How To Know if You Have Sjogren's Syndrome

Many people can have some form of dry eyes for many reasons, including living in an arid environment, taking certain medications (such as antihistamines for allergies and antiarrhythmics for heart regulation), wearing contact lenses or experiencing an age-related decline in the eye's ability to provide adequate lubrication.

But Sjogren's syndrome may be identified by other symptoms in addition to dry eye that are caused by declining function of glands needed to moisten other parts of the body. These symptoms include:

• Unusual dryness in the vaginal area
• Dry sinuses and frequent sinus infections
• Dry and sore mouth
• Inability to chew and swallow properly
• Dry and sore skin
• Joint and muscle pain without accompanying swelling

Other eye symptoms that can occur with Sjogren's syndrome include blurred vision, a burning sensation, sensation of grittiness and light sensitivity.

Various tests may be needed to confirm a diagnosis of Sjogren's syndrome. A tissue sample (biopsy) for analysis may be taken from a salivary gland of your lip. The extent of your dry eye may be measured with a strip of test paper (Schirmer's test) inserted in the corner of your eye to measure the amount of tears that you produce in a certain period of time, generally about five minutes.

Dry eye may be measured also through vital dye staining, such as rose bengal (magenta dye), lissamine (green dye) or fluorescein (orange dye), with examination of the eye's cornea and conjunctiva.

The amount and quality of saliva produced in your mouth may also be measured. Your doctor may order other related tests, such as blood and urine analysis.

Certain markers in the blood — antinuclear antibodies (ANAs) — are specific for Sjogren's syndrome. These markers, known as SS-A (or SS-Ro) and SS-B (or SS-La) are commonly found in people with Sjogren's.

A simple blood test can detect the presence of these antibodies. However, you can still have Sjogren's even if your blood test result is negative for these antibodies.

Using artificial tears and drinking plenty of water can alleviate symptoms of Sjogren's syndrome. (Eye drop photo: Alcon Inc.)

How Dry Eye From Sjogren's Syndrome Is Treated

Dry eye resulting from Sjogren's syndrome may require ongoing treatment with artificial tears, ointments or other remedies. For pain or inflammation, you may also need to take nonsteroidal anti-inflammatory drugs (NSAIDs) such as aspirin or ibuprofen.

If you have an extremely severe case of Sjogren's syndrome, your doctor may consider drugs such as immunosuppressants.

Side Effects of Sjogren's Syndrome

Because chronic dry eye is one of the major symptoms of Sjogren's syndrome, you must make sure your eyes are lubricated to avoid damage to the surface tissue, including the cornea and conjunctiva, that can lead to scarring and infection. Irritation and inflammation of the eyelid glands can lead to blepharitis.

Because the mouth can become unusually dry, you may experience rapid tooth decay or loss. You may need artificial saliva lubricants in order to moisten your mouth and aid in swallowing.

Also, people with Sjogren's syndrome are more likely to develop lymphoma, or cancer of the lymphatic system, which contains white blood cells that play a major role in fighting disease. Lymph nodes may become enlarged or swollen. Sjogren's syndrome also can lead to vasculitis, which is inflammation and sometimes narrowing of blood vessels.

Pregnant women diagnosed with Sjogren's syndrome should notify their doctors. Certain proteins produced by the immune system (autoantibodies) that are capable of attacking other potentially beneficial proteins in the body can be passed along to infants.

How To Prevent Dry Eye and Other Sjogren's Syndrome Symptoms

While you may be unable to prevent the condition, these steps may help ease symptoms:

• Drink more fluids, especially water.
• Chew sugarless gum or hard candies to moisten your mouth.
• Regularly use artificial tears and ointments to keep your eyes moistened. (Your doctor can advise you about appropriate formulas for your particular condition.)
• Use vaginal lubricants.
• Use saline spray for your nose.
• Humidify the air to improve dry skin.
• Tell your eye doctor about any drugs you are taking, because some, such as antihistamines for allergies, can cause dryness.
• Don't smoke, and avoid alcohol.

If you have Sjogren's syndrome, you may be comforted to know that you are not alone. If you find it challenging to live with more severe symptoms of Sjogren's syndrome, consider finding or establishing a support group in your area.

[Page updated September 2008]