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Definition: Sjogren's syndrome from Merriam-Webster's Medical Dictionary

a chronic inflammatory autoimmune disease that affects especially older women, that is characterized by dryness of mucous membranes especially of the eyes and mouth and by infiltration of the affected tissues by lymphocytes, and that is often associated with rheumatoid arthritis —called also sicca syndrome, Sjögren's, Sjögren's disease

Sjögren, Henrik Samuel Conrad (1899–1986), Swedish ophthalmologist. Sjögren served as a professor of medicine at Lund, Sweden, and eventually held the position of chief physician at the hospital there. He invented ophthalmological instruments. He first described Sjögren's syndrome in 1933.


Summary Article: Sjogren's Syndrome from Harvard Medical School Health Topics A-Z
What Is It?

Sjögren's (pronounced "show grins") syndrome is a chronic (or lifelong) condition that causes dry mouth and dry eyes. The syndrome also can affect any of the body's glands, including those that secrete sweat, saliva and oil.

Sjögren's syndrome is an autoimmune disorder, meaning the body's immune system mistakenly attacks the body's own cells and organs. In this case, the immune system attacks the organs that normally produce lubricating fluid, including the salivary glands in the mouth and the lacrimal glands in the eye. This leads to scarring and, eventually, a marked reduction in tear and saliva production, which causes dryness of the eyes and mouth.

Dry mouth and dry eyes can lead to tooth decay, periodontal disease, poorly fitting dentures, salivary gland stones, infection of the salivary glands, oral fungal infections (thrush), mouth sores, weight loss, malnutrition, bacterial conjunctivitis (a bacterial infection of the conjunctiva), corneal damage and vision loss.

Named after Swedish eye doctor Dr. Henrik Sjögren, this syndrome can affect people of all ages and races. However, 90% of all cases involve women, most commonly between the ages of 45 and 55. The condition affects between 2 million and 4 million people in the United States. The cause of this disease is unknown. Research is ongoing, including recent discoveries that certain genes may make a person more prone to developing Sjögren's syndrome.

About half of people with Sjögren's syndrome also have another connective-tissue disease such as rheumatoid arthritis or systemic lupus erythematosus (SLE or lupus). In these people, Sjögren's syndrome is referred to as a secondary condition. In people without another connective-tissue disease, Sjögren's syndrome is called a primary condition.

Symptoms

Some people experience only minor symptoms. Others have blurred vision, eye discomfort, recurring mouth infections, swollen salivary glands and difficulty swallowing or eating. Sjögren's syndrome also can affect the lungs, joints, vagina, pancreas, kidneys, skin and brain.

Symptoms include eye pain, irritation, or redness, and dry mouth. The eyes can feel dry and sandy. Many people with this condition also have vaginal dryness, joint pain and stiffness, muscle aches, dry, cracked tongue and enlarged lymph glands. Other symptoms depend on what other parts of the body are affected. For example, shortness of breath may develop because of lung inflammation and scarring.

Diagnosis

Sjögren's syndrome may be difficult to diagnose because its symptoms are also characteristic of other diseases. Also, disease manifestations vary markedly among different people with this illness.

If your doctor suspects you may have Sjögren's, blood tests can determine if you have markers (autoantibodies) of the disease. However, these tests are not reliable enough to confirm the diagnosis by themselves. Certain tests can determine the amount of lubrication your body's glands are producing. A Schirmer test uses a small piece of filter paper placed under the lower eyelid. A slit-lamp examination involves placing a drop of dye into the eye and examining the eye with a special instrument. A salivary-function test measures the amount of dryness in the mouth. Your dentist or oral surgeon also may recommend a lip biopsy to examine a sample of the minor salivary glands. In a biopsy, a small piece of tissue is removed and examined in a laboratory.

Expected Duration

Sjögren's syndrome is generally a lifelong disease.

Prevention

There is no known way to prevent Sjögren's syndrome, although you can prevent symptoms from getting worse by avoiding certain medications, dry environments, caffeine and alcohol. Complications, such as cavities, may be prevented as well.

Treatment

Sjögren's syndrome cannot be cured, but proper treatment can help to relieve symptoms and allow you to live more comfortably. Aspirin and nonsteroidal anti-inflammatory medications (NSAIDs) can reduce joint swelling and stiffness, and muscle aches. Your physician may prescribe corticosteroids or immunosuppressive drugs for more serious complications. Discuss with your doctor whether any other medications you are taking, such as antihistamines or antidepressants, may be contributing to dryness.

Ask your physician or ophthalmologist for a good-quality artificial-tear preparation or lubricating ointment. Moisture-chamber eyeglasses, which preserve existing tears and protect the eyes from drafts and wind, also can be useful. When eye dryness is severe, a procedure called punctal occlusion (with punctal plugs) may be performed. This procedure plugs the tiny drain holes in the inner corner of the eye. This slows down tear drainage, encouraging tears to accumulate and moisten the eye. An immune suppressing eye drop, cyclosporine, may be useful and is one of the newer medications approved for this condition. A newer treatment for dry eyes is a special contact lens (called a scleral lens) that is larger than standard contact lenses.

Lubricants also are available for nasal and vaginal dryness. A good moisturizer can alleviate the tightness of dry skin. Humidifiers increase the moisture in the home or office. Avoid drafts from air conditioners, heaters and radiators when possible.

To ease dry mouth, drink plenty of liquids throughout the day. Sugarless gum and candy can stimulate saliva production. Artificial-saliva preparations also are available. Avoid caffeinated beverages and alcohol because they can increase dryness. Also avoid acidic or spicy foods, which can irritate your mouth. In severe cases, your dentist or physician may prescribe medications such as pilocarpine (Salagen) or cevimeline (Evoxac). These tend to work better to relieve dry mouth than dry eyes. When Sjögren's syndrome is accompanied by joint inflammation, medications used to treat rheumatoid arthritis (such as hydroxychloroquine or methotrexate) may be helpful. Limited studies suggest that injections of interferon or rituximab may reduce symptoms of Sjögren's syndrome that do not respond to other approaches.

Brush your teeth immediately after eating with toothpaste that contains fluoride, and see your dentist frequently. You have a higher risk of tooth decay because you have less saliva, which has antibacterial properties that help protect your teeth, and also washes the surfaces of your teeth. Don't smoke or use other forms of tobacco. They can harm your mouth, nasal tissue, eyes and lungs. Fluoride and anti-bacterial rinses (such as chlorhexidine-fluoride) may be helpful to prevent dental and periodontal disease.

Walking or swimming can help keep your joints and muscles flexible. If Sjögren's syndrome is a secondary symptom of a connective-tissue disease, medications and the type and intensity of your exercise program should be tailored to that specific disease.

When To Call a Professional

If you have dry eyes and mouth for more than a few days, call your doctor or dentist. Keep in mind that Sjögren's syndrome is difficult to diagnose, and your symptoms may be due to another condition.

Prognosis

The prognosis is highly variable. Some people may experience only mild symptoms and be able to control them with medications. Others can cycle through periods of relative healthiness followed by severe disease. Although there is an increased risk of lymphoma (a cancer of the lymph nodes) in this disease, most people with Sjögren's syndrome have a normal life span and good quality of life.

Additional Info

Sjögren's Syndrome Clinic

The National Institute of Dental and Craniofacial Research

National Institutes of Health

Bethesda, MD 20892-2190

Phone: 301-496-4261

www.nidcr.nih.gov/Research/NIDCRLaboratories/MolecularPhysiology/SjogrensSyndrome/

American College of Rheumatology

1800 Century Place

Suite 250

Atlanta, GA 30345-4300

Phone: 404-633-3777

Fax: 404-633-1870

http://www.rheumatology.org/

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Shmerling, Robert H. (HMFP - Rheumatology)
Copyright 2016 Harvard Health Publications

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