Sjögren's Syndrome

Sjögren's (pronounced SHOW-grins) syndrome is an autoimmune disease. That means the immune system (Read about "The Immune System") attacks the body's own cells. In Sjögren's syndrome, the immune system targets moisture-producing glands such as the salivary glands and the tear-producing glands resulting in dryness in the mouth and eyes. (Read about dry mouth in "Oral Health" "Dry Eye") The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) says other parts of the body can be affected as well, resulting in a wide range of possible symptoms, but the main symptoms involve the mouth and the eyes. The medical term for dry eyes is called keratoconjunctivitis sicca, or KCS, and dry mouth is called xerostomia. Your healthcare provider (HCP) may use these terms when talking to you about Sjögren's syndrome.

Experts believe one to 4 million people in the United States have the disease. Most, 9 out of 10, are women, according to the Sjögren's Syndrome Foundation (SSF). It can occur at any age, but it usually is diagnosed after age 40 and can affect people of all races and ethnic backgrounds. It's rare in children, but it can occur.

There are two types of Sjögren's. Primary Sjögren's occurs by itself, and secondary Sjögren's occurs with another disease, according to NIAMS. SSF says that patients are about evenly split between primary and secondary. The most common diseases that exist with Sjögren's, according to SSF are:

• rheumatoid arthritis (Read about "Rheumatoid Arthritis")
• systemic lupus (Read about "Lupus")
• systemic sclerosis (scleroderma) (Read about "Scleroderma")
• polymyositis/dermatomyositis (Read about polymyositis and dermatomyositis in "Neuromuscular Diseases")

NIAMS says there are other diseases and conditions that are often found in conjunction with Sjögren's. They include:

• skin problems (Read about "Skin")
• lung problems such as pneumonia (Read about "Pneumonia")
• vaginal dryness
• kidney problems (Read about "Kidney Disease")
• nerve problems such as carpal tunnel syndrome (Read about peripheral nerve diseases in "Neuromuscular Diseases" "Carpal Tunnel Syndrome")
• digestive issues, particularly involving the liver (Read about "Digestive System" "The Liver")
• Raynaud's phenomenon (Read about "Raynaud's Phenomenon")
• Graves disease (Read about Graves disease in "Thyroid")
• thrush (Read about "Thrush")

Lymphoma (Read about "Lymphoma") is of special concern to Sjögren's patients. NIAMS says that about 5 percent of Sjögren's sufferers develop lymphoma. SSF adds that one study showed that the incidence of lymphoma was 44 times higher in Sjögren's patients.

Diagnosis and testing

There are a number of tests that a doctor can use to diagnose Sjögren's. Blood tests check for antibodies. NIAMS says people with primary disease are more likely to have certain antibodies (substances that help fight a particular disease) circulating in their blood than people with secondary disease. These antibodies are called SS-A and SS-B. SSF says that 70 percent of patients test positive for SS-A and 40 percent for SS-B. People with primary Sjögren's are more likely (about 70 percent of patients test positive, according to SSF) to have antinuclear antibodies (ANAs) in their blood. ANAs are autoantibodies, which are directed against the body.

Common eye and mouth tests, according to NIAMS include:

• Schirmer test - This test measures tears to see how the lacrimal, (tear producing) gland is working.
• Staining with vital dyes (rose bengal or lissamine green) - The tests show how much damage dryness has done to the surface of the eye. The doctor puts a drop of a liquid containing a dye into the lower eyelid. These drops stain on the surface of the eye, highlighting any areas of injury.
• Slit lamp examination - This test shows how severe the dryness is and whether the outside of the eye is inflamed. An ophthalmologist (eye specialist) uses equipment that magnifies to examine the eye.
• Mouth exam - The doctor will look in the mouth for signs of dryness and to see whether any of the major salivary glands are swollen. Signs of dryness include a dry, sticky mouth; cavities; thick saliva, or none at all; a smooth look to the tongue; redness in the mouth; dry, cracked lips; and sores at the corners of the mouth. The doctor might also try to get a sample of saliva to see how much the glands are producing and to check its quality.
• Salivary gland biopsy of the lip - This test is the best way to find out whether dry mouth is caused by Sjögren's syndrome. The doctor removes tiny minor salivary glands from the inside of the lower lip and examines them under the microscope. If the glands contain lymphocytes in a particular pattern, the test is positive for Sjögren's syndrome. (Read about "Biopsy")

Treatments

Treatment is different for each person, depending on what parts of the body are affected. But in all cases, your HCP will want to help relieve your symptoms, especially dryness. Ask your doctor whether any of your medications contribute to dryness and, if so, how to reduce that effect. Drugs that can cause dryness include:

• antihistamines
• decongestants
• diuretics
• some antidiarrhea drugs (Read about "Diarrhea")
• some antipsychotic drugs
• tranquilizers
• some blood pressure medicines (Read about "Hypertension: High Blood Pressure")
• antidepressants (Read about "Depressive Illnesses")

You can also use artificial tears to help with dry eyes and saliva stimulants and mouth lubricants for dry mouth.

Oral hygiene becomes very important. Natural saliva contains substances that rid the mouth of the bacteria that cause cavities and mouth infections (Read about "Microorganisms"), so good oral hygiene (Read about oral hygiene in "Oral Health") is extremely important when you have dry mouth. Here's what you can do to prevent cavities and infections, according to NIAMS:

• Visit a dentist at least three times a year.
• Rinse your mouth with water several times a day. Don't use mouthwash that contains alcohol because alcohol is drying.
• Use fluoride toothpaste to gently brush your teeth, gums, and tongue after each meal and before bedtime. Non-foaming toothpaste is less drying.
• Floss your teeth every day.
• Avoid sugar. That means choosing sugar-free gum, candy and soda. If you do eat or drink sugary foods, brush your teeth immediately afterward.
• Look at your mouth every day to check for redness or sores. See a dentist right away if you notice anything unusual or have any mouth pain or bleeding.
• Ask your dentist whether you need to take fluoride supplements, use a fluoride gel at night, or have a protective varnish put on your teeth to protect the enamel.

When it comes to your eyes there are many things you can do as well.

• Don't use artificial tears that irritate your eyes.
• Non-preserved drops may be more comfortable.
• Blink several times a minute while reading or working on the computer.
• Protect your eyes from drafts, breezes and wind.
• Put a humidifier in the rooms where you spend the most time, including the bedroom, or install a humidifier in your heating and air conditioning unit.
• Don't smoke and stay out of smoky rooms.
• Apply mascara only to the tips of your lashes so it doesn't get in your eyes. If you use eyeliner or eye shadow, put it only on the skin above your lashes, not on the sensitive skin under your lashes, close to your eyes.
• Ask your doctor whether any of your medications contribute to dryness and, if so, how to reduce that effect.

The cause or causes of Sjögren's syndrome remain under investigation. NIAMS says that it may be a combination of genetics and environmental triggers.