Sjögren's Syndrome

Topic Overview

What is Sjögren's syndrome?

Sjögren's syndrome (say "SHOW-grins") is a disorder in which the immune system attacks the body's moisture-producing glands, such as the tear glands and the saliva glands. These glands may become scarred and damaged, and extreme dryness in the eyes and mouth (sicca syndrome) may develop. Sjögren's syndrome may also cause fatigue, pain in the joints, and, in rare cases, problems with the function of vital organs, such as the lungs, kidneys, and nerves.

Sjögren's syndrome is an autoimmune disease, in which a person's immune system attacks its own tissues as though they were foreign substances. Sjögren's affects over one million people throughout the United States and is most common in white women who are in their 40s and 50s. Women and men of all races may develop this disorder, but nine times more women than men have Sjögren's syndrome.¹

What causes Sjögren's syndrome?

The exact cause of Sjögren's syndrome is not known. A combination of genetic and environmental factors may cause this disorder. Heredity may be a factor, since Sjögren's syndrome sometimes runs in families. Hormones may play a role, since this disorder mostly affects women, but this relationship remains unclear.

What are the symptoms?

The most common symptoms of Sjögren's syndrome are extremely dry eyes and mouth that have persisted for at least 3 months and are not due to medicines. You may have itching and burning in your eyes, and your mouth may feel as though it is full of cotton.

Sjögren's syndrome may develop in a person who has a connective tissue disorder, such as rheumatoid arthritis, lupus, or scleroderma, and is then classified as secondary Sjögren's syndrome. Symptoms of primary (no other associated disorder) and secondary Sjögren's syndrome are the same.

The focus of this topic is primary Sjögren's syndrome.

How is Sjögren's syndrome diagnosed?

Your doctor may diagnose Sjögren's syndrome after taking your medical history, doing a physical examination, and noting symptoms of dry eyes and mouth. Next, he or she will ask whether you are taking any medicines such as antihistamines or antidepressants that could cause dry eyes and mouth. If needed, your doctor may order tests for tear flow, eye damage, saliva production, tissue damage, and blood antibodies to confirm a diagnosis of Sjögren's syndrome.

How is it treated?

Although there currently is no cure for Sjögren's syndrome, you and your doctor can work together to treat your symptoms. For example, you can use artificial tears to moisten your eyes, and you can use mouth lubricants and saliva substitutes to moisten your mouth and throat.

Frequently Asked Questions

Learning about Sjögren's syndrome:	What is Sjögren's syndrome? What causes Sjögren's syndrome? What are the symptoms of Sjögren's syndrome? How common is Sjögren's syndrome? When do I need to see my doctor for Sjögren's syndrome?
Being diagnosed:	How is Sjögren's syndrome diagnosed?
Getting treatment:	How is Sjögren's syndrome treated? What medications will I need to take? What type of doctor should I see for Sjögren's syndrome?
Ongoing concerns:	How will my Sjögren's syndrome progress?
Living with Sjögren's syndrome:	What lifestyle changes should I make? How can I keep my eyes and mouth moist?

Symptoms

The most common symptoms of Sjögren's syndrome are extremely dry eyes and mouth that have lasted for at least 3 months and are not due to medicines. You may have itching and burning in your eyes, and your mouth may feel as though it is full of cotton.

**Dry eyes and dry mouth (sicca syndrome)**
Dry eyes:	Dry mouth:
Dry, gritty, sandy, or itchy feeling in your eyes Thick, ropelike strands of dried mucus in your eyes when you wake up Redness and less tearing A "filmy" feeling in your eyes that interferes with vision Bright lights may bother your eyes Inflamed eyelids (blepharitis) Eye fatigue	Less saliva than usual Trouble swallowing food without also drinking a liquid Abnormal sense of taste Sores (fissures) on the tongue and lips Many cavities and gum disease caused by rapid tooth decay Decreased sense of taste and smell Mouth may feel full of cotton

People with Sjögren's syndrome may also notice:

Dry nose and throat, which can lead to sinus infections, bronchitis, and pneumonia.
Skin problems such as rashes that may be sensitive to light, or dry skin with less sweat production.
Yeast infections of the mouth (thrush) or skin.
Vaginal dryness, causing discomfort, itching, and painful intercourse.
Fatigue and joint or muscle pain.
Pain and difficulty with dentures, which may lead to dental restorations.
Cold, numb, painful fingers and hands (Raynaud's phenomenon).

Sjögren's syndrome may develop in a person who has a connective tissue disorder, such as rheumatoid arthritis, lupus, or scleroderma. In these cases, it is called secondary Sjögren's syndrome. Symptoms of primary (no associated disorder) and secondary Sjögren's syndrome are the same. But it may be difficult to tell the difference between symptoms caused by secondary Sjögren's syndrome and those caused by another connective tissue disorder.

If you have symptoms other than or along with those identified, it is possible you may have another autoimmune disease in addition to Sjögren's syndrome. Discuss all of your symptoms with your doctor so that you can be accurately diagnosed.

Progression of Sjögren's syndrome varies by individual. Some people develop symptoms in other parts of the body, while about 12% spontaneously improve. Most people with Sjögren's syndrome have chronic dryness of the eyes and mouth that stays with them throughout their lives.¹

While not common, serious complications involving major body organs (such as the lungs, nerves, kidneys, and liver) may develop as the disease progresses. A small percentage of people with Sjögren's have a slightly higher chance of developing cancer involving the lymphatic system, such as non-Hodgkin's lymphoma.²

There are many other conditions with similar symptoms. Your doctor will review your symptoms and do laboratory tests to confirm a diagnosis of Sjögren's syndrome.

Who to See

Sjögren's syndrome can affect many different body systems. Doctors in several specialties can diagnose this disorder. Depending on your symptoms, you may want to work with several doctors:

Exams and Tests

After taking your medical history and doing a physical exam, your doctor will assess your symptoms and run certain laboratory tests to confirm a diagnosis of Sjögren's syndrome. He or she will ask you for a list of the medicines you take to determine whether they may be causing your symptoms or making your symptoms worse.

For consistent treatment of Sjögren's syndrome, criteria devised by a group of European doctors are used to help diagnose cases of this disease and to identify cases in which a person has similar symptoms but has other medical problems that may be causing the symptoms. The following tests may help find inflammation of saliva gland tissue, decreased formation of tears or saliva, or certain substances in the blood:

Schirmer's tests measure tear flow. In Schirmer I, a small strip of soft paper is placed in your lower eyelid to absorb your tear flow. Your doctor then measures the area of the paper that is moistened, to evaluate the amount of tear flow. In Schirmer II, a cotton swab is placed gently into your nose to stimulate tear production, in order to measure how fast tears form in your eyes when you feel an irritation in your nose. One or both of these tests will help determine whether your tear (lacrimal) glands are responding properly.
Rose Bengal tests measure potential damage to eyes. A small drop of dye is placed in your lower eyelid. The dye coats the surface of your eye and is then rinsed out. A slit lamp is then used to study the surface of the eye to look for dry or scratched areas. This test is usually performed by an ophthalmologist.
Salivary function tests measure the amount of saliva you produce. A straw is placed in your mouth, which pools the saliva into a cup. Decreased saliva production is often present if you have Sjögren's syndrome.
Blood tests for Sjögren's syndrome determine whether autoantibodies are present in your blood. The presence of autoantibodies, which are proteins produced by the immune system, may help your doctor diagnose Sjögren's syndrome.
Tissue biopsies are done to see whether the salivary gland tissue is inflamed. A small tissue sample (biopsy) is removed from a small salivary gland on the inside of your mouth.
Nuclear medicine scans of the salivary glands are done to see whether the glands appear to be normal.

Your doctor may do other tests to check for evidence of other autoimmune diseases that also may cause dry eyes and mouth and other symptoms not related to Sjögren's. These tests include:

Erythrocyte sedimentation rate, to find out whether a condition causing inflammation is present.
Rheumatoid factor, to help support a diagnosis of rheumatoid arthritis.
Antinuclear antibodies, to help diagnose systemic lupus erythematosus (SLE) and other suspected autoimmune diseases.

Treatment Overview

There is no cure for Sjögren's syndrome. But treatment can help control your symptoms. Treatment options include not only medicines you can take to supplement tears and saliva but also things you can do at home to prevent eye damage and dental problems.

Initial treatment

You can control your symptoms of Sjögren's syndrome, which are often distressing but are rarely disabling. Your doctor may suggest home treatment to:

Provide moisture to your eyes and mouth, by using artificial teardrops and artificial saliva.
Prevent eye damage, by protecting your eyes from wind, smoke, and other irritants.
Prevent dental problems, by brushing and flossing your teeth.
Prevent fatigue, by balancing rest and exercise.
Relieve respiratory and skin problems, by humidifying your home and office and using moisturizing creams.
Relieve acid reflux by taking antacids, such as ranitidine (Zantac), cimetidine (Tagamet), or omeprazole (Prilosec).
Replenish vaginal moisture.
Control pain with gentle exercise and acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs).

Ongoing treatment

Progression of Sjögren's syndrome varies by individual. Most people with this disease have chronic dryness of the eyes and mouth that lasts throughout their lives. Your doctor may suggest home treatment to:

Provide moisture to your eyes and mouth, by using artificial teardrops and artificial saliva.
Prevent eye damage, by protecting your eyes from wind, smoke, and other irritants.
Prevent dental problems, by brushing and flossing your teeth.
Prevent fatigue, by balancing rest and exercise.
Relieve respiratory and skin problems, by humidifying your home and office and using moisturizing creams.
Relieve acid reflux by taking antacids, such as ranitidine (Zantac), cimetidine (Tagamet), or omeprazole (Prilosec).
Replenish vaginal moisture.
Control pain with gentle exercise and acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs).

If your symptoms fail to improve or get worse even with home treatment, your doctor may want to prescribe medicines or use other treatments.

For dry eyes: Your doctor may prescribe:
- Cevimeline (Evoxac) capsules or pilocarpine (Salagen) tablets to stimulate tear production. These medicines are usually prescribed for dry mouth, but recent studies report that they may be effective in relieving dry eyes also.
- Topical antibiotics if you develop redness, swelling, and pain of the eyelids (blepharitis).
For dry mouth: Your doctor may:
- Recommend that you use artificial saliva products to coat the mouth, and/or place sugarless lozenges under the tongue to stimulate saliva production.
- Prescribe antifungal medicines if a yeast infection such as thrush develops in your mouth. For more information, see the topic Thrush.
- Recommend a fluoride rinse or brush-on topical fluoride varnish, such as Colgate Duraphat or Prevident Prophylaxis Paste, to help prevent cavities caused by rapid tooth decay.³
- Prescribe a saliva stimulant, such as pilocarpine tablets or cevimeline capsules.
- Recommend that you avoid antihistamine medicines, which can make dry mouth worse.
For vaginal dryness: Your doctor may prescribe the hormone estrogen in either topical creams doses if nonprescription vaginal moisturizers and lubricants have not relieved dryness and painful intercourse.

Treatment if the condition gets worse

Progression of Sjögren's syndrome is different for everyone. Most people with this disease have chronic dryness of the eyes and mouth that lasts throughout their lives. If your symptoms are not relieved by home treatment and medicines and your disease begins to affect other parts of the body, your doctor may prescribe stronger medicine or recommend surgery.

If extremely dry eyes are not helped by tear substitutes, topical cyclosporine ophthalmic eyedrops (Restasis) may provide relief.

If neither tear substitutes nor cyclosporine eyedrops ease your dry eyes, your doctor may perform a surgical procedure called punctal occlusion, in which he or she places temporary or permanent plugs in your tear ducts (lacrimal ducts) to help keep moisture in your eyes. These plugs keep your tears from draining away from the eyes and leaving them dry.

For joint pain, chronic inflammation in saliva and tear glands, or other serious symptoms, treatment may include:

Nonsteroidal anti-inflammatory drugs (NSAIDs). NSAIDs may provide relief from inflammation, but people with Sjögren's syndrome may be more susceptible to developing gastroesophageal reflux disease (GERD) after taking NSAIDs.
Corticosteroids (such as prednisone), which are used to relieve muscle and joint inflammation but can have serious side effects, including osteoporosis, glaucoma, and diabetes.
Disease-modifying antirheumatic drugs (DMARDs), also known as slow-acting antirheumatic drugs (SAARDs). These medicines may be prescribed alone or in combination to manage the symptoms of joint and muscle pain and dry skin from Sjögren's syndrome. DMARDs that may be prescribed include hydroxychloroquine sulfate (Plaquenil) or methotrexate.

What to think about

Research to develop new medicines to treat the symptoms of Sjögren's syndrome is ongoing.

Interferon alfa (IFN-alfa) may help increase your production of saliva.¹ Experts are uncertain about the benefit of this treatment.
Medicines that modify immune system function, such as infliximab, are being studied to see whether they can reduce glandular inflammation in Sjögren's syndrome.

Home Treatment

Sjögren's syndrome is a disease that causes dry eyes and dry mouth. The following steps and treatments can be very helpful in relieving your symptoms and improving the quality of your life. Also, getting plenty of rest, eating well, and doing mild exercise daily play an important role in successful home treatment of this condition.

Eyes

Use artificial teardrops throughout the day. Artificial tears come in different formulas, so if one type does not help, try another. Try to use preservative-free drops, which are less irritating to the eyes. Artificial tears are available in single-dose packets, which help to avoid bacterial contamination.
Use lubricating ointments at night. Lubricants are thicker and last longer than artificial tears, so there is less burning, dryness, and itching when you wake up in the morning. Be aware that nighttime lubricants may temporarily blur your vision when you first apply them.
Avoid medicines that are known to cause dry eyes, such as antidepressants, antihistamines, and diuretics.
Protect your eyes from wind, breeze, and drafts.
Avoid smoke.
Keep eye makeup away from your eyes.
Use wraparound sunglasses to better protect your eyes from the sun, wind, and grit.

Mouth

Drink fluids throughout the day to keep your mouth moist. Keep water by your bedside at night. But be aware that drinking large amounts of water does not reduce mouth dryness and causes excessive urination during the night. Try drinking small sips of water and rinsing your mouth frequently. Sucking on ice chips can also help.
Use artificial saliva substitutes (mouthwash or spray), which coat the mouth.
Avoid medicines that are known to cause a dry mouth, such as antidepressants, antihistamines, and diuretics.
Brush your teeth twice a day and after meals with fluoride toothpaste, and floss your teeth every day.
Make frequent visits to the dentist to prevent and treat tooth decay.
Use antifungal medicines to treat thrush, a yeast infection that develops in the mouth.
Use sugar-free gum or candies such as lemon drops that naturally stimulate saliva production. (Sugar can increase your risk for cavities and yeast infections.)

Skin

Use moisturizing skin creams or ointments throughout the day.
Shower instead of taking a bath. Use only moisturizing soaps.
After showering, pat excess water off, leaving the skin moist. Then, replenish the moisture in your skin by applying a skin cream or ointment.
Your skin may be extra-sensitive to the sun. Avoid the midday sun, from about 10 a.m. to 4 p.m. Cover your skin when you are outside—for example, wear long pants and long sleeves and wide-brimmed hats—and use SPF 30 or stronger sunscreen. Many experts recommend using sunscreen with SPF 50. For more information, see the Prevention section of the topic Sunburn.

Respiratory tract

Place a humidifier (and an air purifier, if you feel it helps) in your home and at work to increase your comfort.
Use nasal spray made of water and salt (saline) to help a dry nose or nasal congestion.

Vagina

It is common for women with Sjögren's syndrome to experience vaginal dryness and painful intercourse.

Vaginal moisturizing products help to replenish natural moisture and relieve discomfort. These products include:

Replens, a nonhormonal vaginal moisturizer that lasts for hours or even days.
K-Y Silk-e.
Vagisil Personal Moisturizer.

Vaginal lubricants can make intercourse more comfortable for you by relieving the friction you might experience if you have vaginal dryness. But vaginal lubricants do not add moisture to the vagina and are not useful for everyday moisturizing. Look for a water-based lubricant instead of an oil-based lubricant, which can interfere with the vagina's natural cleansing process. Vaginal lubricants include:

Astroglide.
Wet Lubricant Gel.
K-Y Jelly.
Maxilube.
Surgilube.

Stomach

Take a nonprescription antacid or acid reducer, such as Pepcid AC or Zantac 75, when needed, to reduce heartburn.
Raise the head of your bed 6 inches to reduce the backflow (reflux) of stomach acid into your esophagus when you sleep.
See your doctor if you have heartburn or reflux that does not respond to self-care.

Energy (reducing fatigue)

Listen to your body. Alternate rest with exercise. Gradually doing more exercise may help lower your fatigue.
Limit medicines that might make you feel sleepy, such as those used to treat anxiety, colds, or pain. But do not stop or change your medicine usage before talking with your doctor.
Don't skip meals, especially breakfast. Improving your diet may increase your energy level.
Reduce your use of caffeine, nicotine, and alcohol, which tend to contribute to fatigue.
Cut back on watching television, and instead spend time with friends, try new activities, or travel to break the fatigue cycle.
Get a good night's sleep. This may significantly reduce fatigue.

Your fatigue may be related to another health problem. Sjögren's syndrome may increase your risk of thyroid problems (hypothyroidism or hyperthyroidism) and depression. Effective treatments for these health problems are available. You should see your doctor if you feel persistent fatigue.

Comfort (relieving inflammation and pain)

Try daily gentle exercise—swimming in a warm pool may be good if your joints ache—and get plenty of rest every night to relieve aches.
Acetaminophen and nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin or ibuprofen, can help reduce mild swelling and pain. See a doctor for severe swelling and pain in the glands, joints, and muscles, which may require a different medicine or further evaluation.

Medication safety

Several types of medications can cause dryness or allergic reactions and can make your symptoms worse. Talk to your doctor if you are taking any of the following medicines, but continue to take them unless advised to stop by your doctor:

Antihistamines
Bladder medicines that are not antibiotics
Muscle spasm medicines
Blood pressure medicines
Antidepressants
Decongestants
Diuretics
Heart medicines
Parkinson's disease medicines

Other treatments that may help

More research is needed to determine the benefits and risks of these alternative treatments for Sjögren's syndrome.

Acupuncture. Some studies suggest that acupuncture might offer some improvement in dry eyes and dry mouth symptoms.¹
Evening primrose. The oil of the evening primrose is a source of omega-3 fatty acids, whose mild anti-inflammatory properties may relieve the symptoms of dry eyes and dry mouth.

Before using any treatment for your symptoms, consider the risks and benefits. If you decide to use an alternative medicine or supplement, follow these precautions.

Talk with your doctor before taking an alternative medicine or supplement, especially if you are pregnant or trying to become pregnant, take prescription medicines, or have another health problem.
As with all conventional medicines and supplements, it is important to follow the directions on the label.
Do not exceed the maximum recommended dose.

For more information on alternative medicines and supplements, see the topic Complementary Medicine.

Other Places To Get Help

Organizations

National Institute of Neurological Disorders and Stroke
P.O. Box 5801
Bethesda, MD 20824
Phone:	1-800-352-9424 (301) 496-5751
TDD:	(301) 468-5981
Web Address:	www.ninds.nih.gov

The National Institute of Neurological Disorders and Stroke (NINDS), a part of the National Institutes of Health, is the leading U.S. federal government agency supporting research on brain and nervous system disorders. It provides the public with educational materials and information about these disorders.

American Academy of Ophthalmology (AAO)
P.O. Box 7424
San Francisco, CA 94120-7424
Phone:	(415) 561-8500
Fax:	(415) 561-8533
Web Address:	www.aao.org

The American Academy of Ophthalmology (AAO) is an association of medical eye doctors. It provides general information and brochures on eye conditions and diseases and low-vision resources and services. The AAO is not able to answer questions about specific medical problems or conditions.

American Autoimmune Related Diseases Association (AARDA)
22100 Gratiot Avenue
East Detroit, MI 48021
Phone:	(586) 776-3900
E-mail:	aarda@aol.com
Web Address:	www.aarda.org

This national nonprofit health agency is dedicated to curing autoimmune diseases and relieving the suffering and the economic and social impact of autoimmune diseases. AARDA brings professionals together in the areas of education, research, and patient services, and sponsors physicians' conferences, research, legislative advocacy, and a national awareness campaign.

American College of Rheumatology
1800 Century Place
Suite 250
Atlanta, GA 30345
Phone:	(404) 633-3777
Fax:	(404) 633-1870
Web Address:	www.rheumatology.org

The American College of Rheumatology (ACR) and the Association of Rheumatology Health Professionals (ARHP, a division of ACR) are professional organizations of rheumatologists and associated health professionals who are dedicated to healing, preventing disability from, and curing the many types of arthritis and related disabling and sometimes fatal disorders of the joints, muscles, and bones. Members of the ACR are physicians; members of the ARHP include research scientists, nurses, physical and occupational therapists, psychologists, and social workers. Both the ACR and the ARHP provide professional education for their members. The ACR Web site offers patient information fact sheets about rheumatic diseases, about medicines used to treat rheumatic diseases, and about care professionals.

National Eye Institute, National Institutes of Health
Information Office
31 Center Drive MSC 2510
Bethesda, MD 20892-2510
Phone:	(301) 496-5248
E-mail:	2020@nei.nih.gov
Web Address:	www.nei.nih.gov

As part of the U.S. National Institutes of Health, the National Eye Institute provides information on eye diseases and vision research. Publications are available to the public at no charge. The Web site includes links to various information resources.

Sjögren's Syndrome Clinic
National Institute of Dental and Craniofacial Research, National Institutes of Health
Bethesda, MD 20892-2190
Phone:	(301) 402-7364
Fax:	(301) 480-4098
E-mail:	nidcrinfo@mail.nih.gov
Web Address:	www.nidcr.nih.gov/Research/Intramural/Molecular_Physiology/SjogrensSyndrome/SjogrensSyndromeClinic.htm

This clinic conducts basic and clinical research on salivary function and Sjögren’s syndrome and is located in the Molecular Physiology and Therapeutics Branch, National Institute of Dental and Craniofacial Research (NIDCR), National Institutes of Health (NIH) in Bethesda, Maryland.

Sjögren's Syndrome Foundation
6707 Democracy Boulevard
Suite 325
Bethesda, MD 20817
Phone:	1-800-475-6473
Fax:	(301) 530-4415
Web Address:	www.sjogrens.com

This nonprofit foundation educates both health professionals and people with Sjögren's syndrome about the disease. It also produces and distributes educational materials, including the Moisture Seekers newsletter, handbooks, videos, and medical articles. A network of chapters, support groups, and contacts organizes meetings, participates in health fairs, increases awareness in their community, and promotes local media campaigns. The foundation sponsors educational programs, symposia, and workshops; provides physician and dentist referrals; and advocates for research and treatment centers.

Related Information

References

Citations

Jonsson R, et al. (2005). Sjögren's syndrome. In WJ Koopman, LW Moreland, eds., Arthritis and Allied Conditions: A Textbook of Rheumatology, 15th ed., vol. 2, pp. 1681–1705. Philadelphia: Lippincott Williams and Wilkins.

Carsons S (2005). Sjögren's syndrome. In ED Harris Jr et al., eds., Kelley's Textbook of Rheumatology, 7th ed., vol. 2, chap. 69, pp. 1105–1124. Philadelphia: Elsevier Saunders.

Fox RI, et al. (2000). Update in Sjögren syndrome. Current Opinion in Rheumatology, 12(5): 391–398.

Credits

Author	Shannon Erstad, MBA/MPH
Editor	Kathleen M. Ariss, MS
Associate Editor	Pat Truman, MATC
Primary Medical Reviewer	Martin Gabica, MD - Family Medicine
Specialist Medical Reviewer	Stanford M. Shoor, MD - Rheumatology
Last Updated	May 6, 2008

Topic Contents