Figure 2 - Salivary glands may become swollen

Figure 1 - Areas that may be affected by Sjogren's syndrome

About

Basics of sjogren's syndrome

Sjogren's syndrome is a chronic autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva. The hallmark symptoms of the disorder are dry mouth and dry eyes. It is named after the Swedish eye doctor, Dr. Henrik Sjogren, who first described it.

Sjogren's syndrome can occur in two ways: primary and secondary. Both forms affect roughly an equal number of people.

Primary Sjogren's syndrome occurs by itself and is not associated with other diseases.

Secondary Sjogren's syndrome occurs with rheumatic diseases such as rheumatoid arthritis, systemic lupus erythematosus ("lupus"), polymyositis, and some forms of scleroderma. Rheumatic diseases are conditions that affect joints, bones, muscles, skin, and sometimes other organs.

Prognosis

Fertility and pregnancy

Incidence

Symptoms

Sjogren's syndrome affects everyone differently. You may not have every symptom listed here, and you may have only minor problems with those you do have. The symptoms may seem worse at some times than at others.

In people with no other health problems, the most common early symptom is the onset of severe dry mouth and eyes.

In people with rheumatoid arthritis or related conditions, dry eyes and mouth develop more slowly. In this case, Sjogren's may be difficult to diagnose.

Symptoms may include:

Dry mouth

The mouth normally contains saliva, which aids chewing and swallowing. In people with Sjogren's syndrome, the amount of saliva is much less. This makes chewing, swallowing, and speaking difficult. It may also cause a decreased sense of taste.

Dry eyes

Your eyes may feel dry, "gritty," or "sandy." They may burn and look red. A thick substance may accumulate in the inner corner of your eyes while you sleep. Your eyes may be more sensitive to sunlight. If not properly treated, Sjogren's syndrome can lead to ulcers of the cornea (the clear covering of the eyeball). On rare occasions, this can cause blindness.

Swollen salivary glands

There are three set of glands that produce saliva. They're located under your tongue, in the cheeks in front of your ears, and in the back of your mouth. They may feel swollen and tender (see figure 2). This may occur along with a fever. This affects about one-half of people with the disorder.

Dental cavities

This is a common problem that results from a dry mouth. Saliva fights bacteria and defends against cavities. Because you have decreased saliva, your teeth may develop cavities more easily.

Dry nose, throat, and lungs

This may make your throat feel dry and tickly. You may have a dry cough, hoarseness, a decreased sense of smell, and nosebleeds. It can also lead to pneumonia, bronchitis, and ear problems.

Dryness of the vagina

This can cause painful intercourse for women with Sjogren's syndrome.

Fatigue

Fatigue is a common complaint. You may get easily exhausted and feel tired and worn out.

Other problems

Sjogren's syndrome can affect other parts of the body, such as blood vessels, the nervous system, muscles, skin, and other organs. This can lead to muscle weakness, confusion and memory problems, dry skin, and feelings of numbness and tingling.

Sjogren's syndrome can also affect the liver and pancreas. When it does, there is a greater chance for developing cancer of the lymph tissue. Although this is unusual, it is one reason why medical exams and continued follow-up are important.

Causes

Effects

In Sjogren's syndrome, changes occur in the immune system--the body's defense against disease. In Sjogren's, the immune system lacks the usual controls. This causes white blood cells to invade glands in the body that produce moisture, such as the tear and salivary glands, and the Bartholins glands in the vagina. They can destroy the glands and cause them to stop producing moisture.

Sjogren's syndrome can also cause problems in other parts of the body, including the joints, lungs, muscles, kidneys, nerves, thyroid gland, liver, pancreas, stomach, and brain (see figure 1). In addition, Sjogren's syndrome may cause skin, nose, and vaginal dryness, and may affect other organs of the body including the kidneys, blood vessels, and lungs. Blindness is a rare complication of Sjogren's.

Diagnosis

Your doctor may do several things to find out if you have Sjogren's syndrome. Some of the tests you may undergo include:

Physical examination

Your doctor will ask you to describe your symptoms, and will look for other symptoms, such as red, itchy eyes; swollen salivary glands; a dry, cracked tongue; and enlarged lymph glands in your neck.

Blood tests

Tests for specific blood markers can determine if you have Sjogren's syndrome. However, not everyone with Sjogren's has these markers.

Diagnostic tests

Schirmer test

This helps determine how dry your eyes may be. It involves placing a small piece of filter paper under the lower eyelid to measure the amount of tears your eyes produce.

Slit-lamp examination

This is a more accurate way to find out if your eyes are dry. In this test, the doctor puts a drop of dye into your eye and examines the eye with a special instrument called a slit lamp. The dye will stain dry or eroded areas of the eye. This test is often done by an ophthalmologist (eye doctor}.

Lip biopsy

In this test, the doctor removes a few salivary glands from inside your lip. The tissue is examined under a microscope. The appearance of the tissue helps determine if you have Sjogren's syndrome.

Salivary function tests

These measure the actual amount of saliva you produce, to help determine if you have Sjogren's.

Urine tests

These may be done to test your kidney function.

Chest X-ray

This can help detect changes in your lungs.

Treatment

As yet, there is no cure for Sjogren's syndrome. However, proper treatment can help relieve symptoms so you can live a comfortable and productive life.

The main goal of treatment is to relieve discomfort and lessen the effects of the dryness. Since Sjogren's syndrome affects everyone differently, your treatment plan will be based on your specific needs.

Self-management

Your treatment may include different ways to relieve your symptoms, such as those listed below. If you have arthritis or another condition, you will also want to follow specific treatment for that condition.

For dry mouth:

• Sip fluids throughout the day.
• Use sugar-free gum or candies to stimulate saliva production.
• Try saliva substitutes or mouth coating products. They may be useful in some people, and are available without a prescription.

To prevent dental cavities:

• Have frequent dental checkups.
• Use mouth rinses that contain fluoride.
• Brush and floss your teeth regularly.
• Use sugar-free products.

For dry eyes:

• Use artificial tears or eye drops to help relieve the discomfort of dry eyes. Use preservative-free products, if you apply the drops more than four times per day.
• Try lubricating ointments or small, long-acting pellets for overnight or long-lasting relief.
• Your ophthalmologist may recommend a simple operation that blocks tear drainage from your eye.

For dry skin:

• Use moisturizing lotions for sensitive skin.
• Avoid drafts from air conditioners, heaters, and radiators, when
• possible.
• Use a humidifier in your house and at work.

For vaginal dryness:

• Use lubricants made specifically to help vaginal dryness. Do not use petroleum jelly.

Exercise and therapy

Medications

Aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs) help reduce joint swelling and stiffness, as well as muscle aches.

If you have serious complications, your doctor may recommend stronger medicines.

Resources

Contact one of the following organizations for more information.

The National Sjogren's Syndrome Association is an international, nonprofit, all-volunteer organization dedicated to providing educational information to patients and health professionals worldwide. It sponsors support groups and national and regional conferences. Publishes a national newsletter ("The Sjogren's Digest"), a quarterly collection of articles ("Patient Education Series"), and a patient guide ("Learning to Live with Sjogren's Syndrome").

National Sjogren's Syndrome Association
Toll-free: 1-800-395-NSSA (6772)

The Sjogren's Syndrome Foundation is an international organization that provides materials, educational programs, and support groups throughout the U.S. and abroad. It publishes "Sjogren's Syndrome Handbook: An Authoritative Guide for Patients" and a monthly newsletter,

Sjogren's Syndrome Foundation
6707 Democracy Blvd Ste 325
Bethesda, MD 20817
tel: (800) 475-6473 fax: (301) 530-4415
www.sjogrens.org

Credits

Some of this material may also be available in an Arthritis Foundation brochure. Contact the Washington/Alaska Chapter Helpline: (800) 542-0295. If dialing from outside of WA and AK, contact the National Helpline: (800) 283-7800.

Adapted from the pamphlet originally prepared for the Arthritis Foundation by Mitchell Friedlaender, M.D. Elaine Harris, Paul F. Howard, M.D. James Sciubba, M.D. John P. Tesser, M.D. and Norman Tallal, M.D. This material is protected by copyright.