5. Sjögren’s Syndrome: Patient Information

Sjögren’s Syndrome

Sjögren’s syndrome is a chronic (lifelong) disorder that causes dry eyes and a dry mouth. It is named after the Swedish eye doctor, Dr. Henrik Sjögren, who first described it.

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

Primary Sjögren’s syndrome occurs by itself and is not associated with other diseases. Secondary Sjögren’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.

In Sjögren’s syndrome, changes occur in the immune system–the body’s defense against disease. In Sjögren’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.

Sjögren’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.

The causes of this condition are not known. There is some evidence that viral infections, heredity, and hormones may in some way contribute to Sjögren’s syndrome.

Sjögren’s syndrome can affect people of any race and any age. It usually affects women. It affects more than one million people in the U.S.

Symptoms

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, Sjögren’s may be difficult to diagnose.

Sjögren’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.

Symptoms include:

• Dry mouth
  The mouth normally contains saliva, which aids chewing and swallowing. In people with Sjögren’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, Sjögren’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 sets 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. 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 Sjögren’s syndrome.
• Fatigue
  Fatigue is a common complaint. You may get easily exhausted and feel tired and worn out.
• Other problems
  Sjögren’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.
  Sjögren’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.

Diagnosis

Your doctor may do several things to find out if you have Sjögren’s syndrome. This includes:

• 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 Sjögren’s syndrome. However, not everyone with Sjögren’s has these markers.
• 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 Sjögren’s syndrome.
• Salivary function tests
  These measure the actual amount of saliva you produce, to help determine if you have Sjögren’s.
• Urine tests
  These may be done to test your kidney function.
• Chest X-ray
  This can help detect changes in your lungs.

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

Treatment

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

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.

See your family doctor and your dentist regularly. Since Sjögren’s syndrome can affect many parts of the body, regular checkups can help detect and prevent future problems. You may also need regular check ups with an arthritis specialist and an eye specialist.

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.

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.

Exercise

Mild exercise, such as walking or swimming, can help keep joints and muscles flexible. Exercise may also protect against further joint damage.

A note about pregnancy: A certain blood marker often found in women with Sjögren’s syndrome can, very rarely, be associated with heart problems in newborn babies. If you’re a woman with Sjögren’s syndrome who is planning to become pregnant, see your doctor about testing for this marker. If it is present, ask your doctor whether pregnancy is advisable. If you do become pregnant, you and your doctor can work out the best plan to manage the situation.

Sjögren’s syndrome is generally not life-threatening. The outlook for people with this condition is usually good. Dryness, however, may last for the rest of your life. By using artificial moisture and practicing good oral hygiene, you can help prevent serious problems.

If you have Sjögren’s syndrome and a rheumatic disease, make sure you follow your doctor’s complete treatment program.

Support and More Information

Contact your local chapter of the Arthritis Foundation for more information about Sjögren’s syndrome. The following booklets may be useful:

• Coping with Pain
• Coping with Stress
• Exercise and Your Arthritis
• Rheumatoid Arthritis
• Scleroderma
• Systemic Lupus Erythematosus

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

National Sjögren’s Syndrome Association
3201 West Evans Drive
Phoenix, AZ 85023
1-800-395-NSSA (6772)

The Sjögren’s Syndrome Foundation is an international organization that provides materials, educational programs, and support groups throughout the U.S. and abroad. Publishes “Sjögren’s Syndrome Handbook: An Authoritative Guide for Patients” and a monthly “Moisture Seekers Newsletter”.

Sjögren’s Syndrome Foundation
366 North Broadway, Suite PH-W2
Jericho, NY 11753
1-800-475-6473