Sjögren's Syndrome

In this condition factsheet:

• The Facts
• Causes
• Symptoms and Complications
• Making the Diagnosis
• Treatment and Prevention

The Facts

Sjögren's syndrome (pronounced "show-grens") occurs when the body's immune system attacks the glands that produce moisture in the mouth, eyes, and other mucous membranes, leading to excessive dryness.

Sjögren's syndrome exists worldwide in people from all backgrounds and age groups. Most people with this condition start to notice symptoms in middle age (mid 40s to 50s), but it can affect people of all ages. Women are 10 times more likely to develop the condition than men. Sjögren's syndrome was first described by an ophthalmologist, Dr. Henrik Sjögren, early in the 20th century.

Treatment for this condition relies on providing relief from these symptoms and, for people with severe cases, suppressing the immune system with medication.

Causes

The exact cause of Sjögren's syndrome is unknown. Some research suggests that genetics along with environmental factors play a role in the development of the condition. Other research suggests that bacterial or viral infections may trigger the immune system to attack the moisture-producing glands.

What we do know is that Sjögren's syndrome is an autoimmune disease in which the body attacks its own healthy cells. The body's immune system begins to destroy cells in the glands that produce saliva, tears, and other fluids in the body. Sjögren's syndrome sometimes begins as a result of another autoimmune disease (e.g., rheumatoid arthritis or lupus), in which case it is classified as secondary Sjögren's syndrome, but it may also develop for no known reason.

Primary Sjögren's syndrome develops in people who have no other autoimmune disease, while secondary Sjögren's is most often identified in people who have lupus or rheumatologic disease (e.g., rheumatoid arthritis).

Symptoms and Complications

Sjögren's syndrome results in a decrease in the production of tears and saliva, and may affect other areas of the body.

Symptoms commonly associated with Sjögren's syndrome include:

• burning sensation in the eyes
• dry, grainy feeling in the eyes
• dry mouth or throat
• difficulty chewing or swallowing
• swollen glands around the face and neck
• increased sensitivity of the eyes to light

Other possible symptoms include:

• vaginal dryness
• dry nasal passages
• dry skin
• joint pain or inflammation
• fatigue
• dry, unproductive cough
• hair loss

In addition to the common symptoms of Sjögren's syndrome, complications may arise. The condition may affect other organs including the blood vessels, brain, kidneys, liver, lungs, and pancreas. There is also a slightly greater risk of lymphoma (cancer of the lymph nodes or saliva glands affected by Sjögren's) in patients with Sjögren's syndrome. As well, dry eyes can increase the risk of eye infections or damage to the cornea, and a dry mouth can increase the risk of cavities and tooth loss.

Making the Diagnosis

Determining whether or not a person has Sjögren's syndrome requires a number of tests and examinations. Blood tests, tear, and saliva production tests as well as tissue samples from the saliva glands and lip may help provide a diagnosis.

Since certain medications (e.g., antihistamines*, decongestants, diuretics, certain antidiarrhea medications, certain antipsychotic medications, tranquillizers, certain blood pressure medications, antidepressants) and conditions may cause symptoms similar to Sjögren's syndrome, doctors must first rule out these causes.

Blood tests can be used to look for the presence of substances (called antibodies) that are common in people with the condition. Rheumatoid factor in the blood may indicate Sjögren's syndrome because it commonly occurs in people with rheumatoid arthritis and may also occur in people with Sjögren's.

Additionally, ultrasounds, MRIs, chest X-rays, and urine samples may also provide clues for a doctor. Affected lungs may appear inflamed, and kidney function may not be at its best in a person with Sjögren's syndrome.

Treatment and Prevention

There is no cure for Sjögren's syndrome. Replacing moisture in the body is the main therapy. Replenishing lost moisture eases some of the symptoms of dryness associated with the condition and improves the quality of life for those affected.

Dry mouth: Treating dry mouth is necessary for many people with Sjögren's syndrome. Doctors often recommend saliva substitutes to make a person's mouth feel moist. This can help with eating and speaking throughout the day. Drinking water and chewing sugarless gum can also help keep the mouth moist. There are also medications available, such as pilocarpine, which stimulates the production of saliva. In addition to making speaking and eating easier, keeping the mouth moist is also important to prevent oral infections such as oral thrush (a fungal infection of yeast in the mouth). Keeping the mouth moist and good dental hygiene can help keep your teeth healthy. If you have Sjögren's syndrome, see your dentist regularly.

Dry eyes: Applying tear drops (artificial tears) or tear gel moistens the eyes and protects the cornea from damage. Moisturizing eye ointments or cyclosporine or lifitegrast eye drops may also be used. In severe cases, the tear duct that drains tears away from the eye may be closed surgically to keep more tears in the eye. Keeping the eyes moist using artificial tears can help prevent eye infections and cornea damage.  Fitted shields on the sides of eyeglasses can be used to protect the eyes from air and wind. Another way to alleviate dry eyes is a simple surgical procedure called punctal occlusion. This procedure is done by inserting small plugs into the tear ducts. Lastly, it's important to see your eye doctor regularly, and get medical attention if you have symptoms such as eye pain or redness, as these may be a sign of eye problems.

Other symptoms: Nonsteroidal anti-inflammatory drugs (NSAIDs) may be recommended to relieve pain associated with joint inflammation due to other autoimmune conditions such as rheumatoid arthritis. In cases that lead to complications throughout the body, medications to suppress the immune system may be necessary.

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