Sjögren's syndrome is a chronic disorder that causes insufficient moisture production in certain glands of the body. It occurs when a person's normally protective immune system attacks and destroys moisture-producing glands, including salivary glands and tear glands. The lungs, bowel, and other organs are  occasionally affected.

Sjögren's syndrome is named after the Swedish eye doctor, Henrik Sjögren, who first described the condition.

Sjögren's syndrome may cause the following symptoms:

• Extremely dry eyes causing a feeling of grit or sand in the eyes or a burning redness.
• Extremely dry mouth and throat causing difficulty with chewing and swallowing, decreased sense of taste, difficulty speaking, an increase in dental cavities, and dry cough or hoarseness.
• Enlarged parotid glands (located at the angle of jaw) and sometimes infection of these glands.
• Excessive fatigue
• Aches and pains in muscles and joints

Less common features of Sjögren's syndrome are:

• Irritation of the nerves in the arms, hands, legs or feet (neuropathy).
• Thyroid gland abnormalities
• Skin rashes
• Memory loss or confusion.
• Numbness or tingling.
• Gastrointestinal problems.
• Inflammation of the lungs, kidneys, liver or pancreas.
• Cancer of the lymphatic tissue (lymphoma) occurs in less than 1% of patients.

What Causes Sjögren's Syndrome?

Normally, the immune system (the body's defense against invaders) protects the body from infection and foreign substances such as bacteria and viruses.

In autoimmune diseases, such as Sjögren's syndrome, the immune system triggers an inflammatory response when there are no foreign substances to fight off. This inflammatory response causes the body's white blood cells to attack and destroy certain moisture-producing glands.

The exact cause for the abnormal immune response in Sjögren's syndrome is unknown. Some theories suggest that a virus or bacteria may alter the immune system, causing it to attack the glands. Some people may have a genetic or inherited factor that makes them more likely to develop Sjogren's syndrome.

What Are the Forms of the Disease?

Sjogren's syndrome occurs in two basic forms: primary Sjögren's syndrome - the disease by itself and not associated with any other illness; and secondary Sjögren's syndrome - disease that develops in the presence of another autoimmune disease such as rheumatoid arthritis, lupus, or vasculitis.

As many as 4 million people in the U.S. have Sjögren's syndrome. More than 90% of these are women. The disease can affect people of any race or age, although the average age of onset is in the late 40s.

How Is Sjögren's Syndrome Diagnosed?

The diagnosis of primary Sjögren's syndrome is based on several factors, including:

• Presence of dry eyes and mouth. An ophthalmologist can detect dry eyes by measuring tear production or carefully examining the cornea of the eye.
• Certain laboratory tests also suggest that dry eyes and mouth are caused by autoimmune mechanisms. Examples include the presence of auto-antibodies in the blood, known as anti-SSA or anti-SSB (also called anti-Ro or anti-La).
• Biopsy of the inner lip (performed in some cases to prove the diagnosis of primary Sjögren's syndrome). The biopsy may show inflammation damaging salivary glands.

Secondary Sjögren's syndrome is generally diagnosed when someone with an established autoimmune disease, such as rheumatoid arthritis or lupus, develops extreme dryness of the eyes and mouth. This diagnosis only rarely requires a lip biopsy.

Can Other Problems Mimic Sjögren's Syndrome?

Sometimes, the use of certain medications can cause side effects that mimic the symptoms of Sjögren's syndrome. Medications such as tricyclic antidepressants (like Pamelor or Elavil) and antihistamines like Benadryl, radiation treatments to the head and neck, as well as other autoimmune disorders, can also cause severely dry eyes and mouth.

How is Sjögren's Syndrome Treated?

There is no cure for Sjögren's syndrome, but it can be treated and controlled. The goals of treatment are to decrease discomfort and reduce the harmful effects of dryness. The type of treatment prescribed will be tailored to each patient's symptoms and needs. The following are some of the main methods of treatment for Sjögren's syndrome.

• Good oral hygiene. Good mouth care may not prevent a dry mouth, but it helps prevent infection. Toothpastes and oral gels are available for people with dry mouth symptoms. These products contain low doses of peroxide. (High amounts can cause more severe dryness.) These products also may have antibacterial action to reduce the severity of dental cavities over a long period of time.

   

• Increasing eye moisture. Dry eyes are mainly treated with the use of artificial tears, and a wide variety of products are available. Artificial tears must be used regularly and more often in dry environmental conditions such as on airplanes, in air-conditioned buildings and on windy days.

  While artificial tears are helpful, they often do not last long enough. Thicker preparations are available that last longer. These often are used at bedtime because they sometimes cause blurry vision. Eye drops containing cyclosporine (Restasis) treat inflammation in the glands around the eyes and may help to increase tear production. Surgery to slow the disappearance of tears by blocking or sealing the tear ducts is another treatment option for more severe cases when artificial tears are not sufficient.

  Surgery to slow the disappearance of tears is another treatment option when artificial tears are not sufficient.

   

• Medications. Medications that tend to deplete body fluids should be avoided. Mild pain-relieving medications including acetaminophen (Tylenol) or Motrin or Aleve can reduce muscle or joint pain. Two prescription medications, Salagen and Evoxac, stimulate saliva production and may relieve the dry mouth symptoms.

  In some patients, the anti-rheumatic drug Plaquenil has been beneficial in decreasing pain and salivary gland swelling.

  For patients with generalized symptoms, particularly when the disease affects internal organs (including the gastrointestinal system, kidneys or brain or spinal cord), high doses of immunosuppressive medications may be necessary. These include medicines such as prednisone (a steroid) and, rarely, chemotherapy-type medications such as methotrexate.

   

• Balance rest and exercise. Guided exercise programs can help patients overcome fatigue, maintain flexibility, and overcome joint and muscle pain.

Reviewed by the doctors at The Cleveland Clinic Department of Rheumatic and Immunologic Diseases

WebMD Medical Reference provided in collaboration with the Cleveland Clinic

SOURCES: Sjogren's Syndrome Foundation. American College of Rheumatology.

Edited by Marc C. Levesque, PhD, MD on October 01, 2006

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