What You Need to Know About Sjögren’s Syndrome

What is Sjögren’s syndrome?
Sjögren’s syndrome is a chronic disorder that causes insufficient moisture production in certain glands of the body.

Sjögren’s syndrome occurs when a person’s normally protective immune system attacks and destroys moisture-producing glands, including salivary (saliva-producing) glands and lacrimal (tear-producing) glands. The lungs, bowel and other organs are less often affected by Sjögren’s syndrome.

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

	Sjögren’s syndrome is characterized by dry eyes and mouth. In some patients, the parotid glands may become visibly enloarged.

What are some symptoms Sjögren’s syndrome may cause?
• Extremely dry eyes causing:
~ Feeling of grit or sand in the eyes
~ Burning
~ Redness

• Extremely dry mouth and throat causing:
~ Difficulty chewing and swallowing
~ Decreased sense of taste
~ Difficulty speaking
~ Increase in dental cavities
~ Dry cough or hoarseness

• Enlarged parotid glands (located at the angle of jaw) and sometimes infection of the parotid glands

• Excessive fatigue

• Aches, 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
• Feeling of numbness or tingling
• Gastrointestinal problems
• Inflammation of the lungs, kidneys, liver or pancreas
• Cancer of the lymphatic tissue (occurs in less than 1% of patients with the disease)

What causes Sjögren’s syndrome?
Normally, the immune system (the body’s defense system) 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. Certain people may have a genetic or inherited factor that makes them more likely to develop Sjögren’s syndrome.

What are the forms of the disease?
Sjögren’s syndrome occurs in two basic forms:
• Primary Sjögren’s syndrome – the disease by itself, not associated with any other illness

• Secondary Sjögren’s syndrome – disease that develops in the presence of another autoimmune disease such as rheumatoid arthritis, systemic lupus erythematosus or vasculitis

Who is affected by the disease?
More than one million people in the United States have Sjögren’s syndrome. Over 90% of people affected by Sjögren’s syndrome are women. The disease can affect people of any race or age.

How is primary Sjögren’s syndrome diagnosed?
The diagnosis of Sjögren’s syndrome is based on several factors, including:

• Presence of dry eyes and mouth
~ Dry eyes can be detected by an ophthalmologist (eye doctor) by measuring tear production or carefully examining the cornea (clear part of the eye).

• Certain laboratory tests also suggest that dry eyes and mouth are caused by autoimmune mechanisms
~ Examples include the presence of autoantibodies in the blood, known as anti-SSA or anti-SSB (also known as 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.

How is secondary Sjögren’s syndrome diagnosed?
Secondary Sjögren’s syndrome is generally diagnosed when someone with an established autoimmune disease such as rheumatoid arthritis or systemic lupus erythematosus 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 mimic the symptoms of Sjögren’s syndrome. Medications such as tricyclic anti-depressants, antihistamines, radiation treatments to the head and neck and 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.

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 could cause worse dryness). These products may also 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 are often used at bedtime because they sometimes cause blurry vision.

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 (analgesics) including acetaminophen (such as Tylenol) or nonsteroidal antiinflammatory drugs (NSAIDs – such as Motrin and Aleve) can reduce muscle or joint pain.

In some patients, the anti-rheumatic drug, hyroxychloroquine, 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 nervous system), high doses of immunosuppressive medications may be necessary. These include medicines such as prednisone (a steroid) and rarely chemotherapy-type medications.

BALANCE OF REST AND EXERCISE
Guided exercise programs can help patients overcome fatigue, maintain flexibility and overcome joint and muscle pain.

How can I learn more about Sjögren’s Syndrome?
The Sjogren's Syndrome Foundation
8120 Woodmont Avenue, Suite 530
Bethesda MD 20814-1437
301-718-0300
1-800-4-SJOGREN (1-800-475-6473)
www.sjogrens.org/
ssf@sjogrens.org

The Cleveland Clinic  |  Contact Us  |  October 2, 2006