Sjögren's Syndrome

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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. Women and men of all races may develop this disorder. But Sjögren's is more common in white women who are in their 40s and 50s.

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:

Being diagnosed:

Getting treatment:

Ongoing concerns:

Living with Sjögren's syndrome:

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 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. When a person has Sjögren's and does not have a connective tissue disorder, it is known as primary Sjögren's syndrome. The symptoms of primary and secondary Sjögren's syndrome are the same. But it may be hard 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.

Most people with Sjögren's syndrome have chronic dryness of the eyes and mouth that stays with them throughout their lives. A few people develop symptoms in other parts of the body.

Though 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.

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. Your family doctor or general practitioner can diagnose this disorder. Depending on your symptoms, you may be referred to a specialist or other health professional:

Examinations and Tests

After taking your medical history and doing a physical examination, your doctor will assess your symptoms and may 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.

Criteria devised by a group of European doctors can be 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:

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, or omeprazole (Losec).
  • Replenish vaginal moisture.
  • Control pain with gentle exercise and acetaminophen or non-steroidal 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, or omeprazole (Losec).
  • Replenish vaginal moisture.
  • Control pain with gentle exercise and acetaminophen or non-steroidal 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:
    • Pilocarpine (Salagen) tablets to stimulate tear production. This medicine is usually prescribed for dry mouth, but studies report that it 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 5000 Plus, to help prevent cavities caused by rapid tooth decay.
    • Prescribe a saliva stimulant, such as pilocarpine tablets.
    • Recommend that you avoid antihistamine medicines or certain older antidepressants, which can make dry mouth worse.
  • For vaginal dryness: Your doctor may prescribe the hormone estrogen in either topical creams doses if non-prescription 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 tear substitutes don't 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:

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 rituximab (Rituxan) and abatacept (Orencia), 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 non-hormonal vaginal moisturizer that lasts for hours or even days.
  • K-Y Silk-E.
  • Vagisil Intimate 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.
  • K-Y Jelly.
  • Taro Lubricating Gel.

Stomach

  • Take a non-prescription antacid or acid reducer, such as Pepcid AC or Zantac 75, when needed, to reduce heartburn.
  • Raise the head of your bed 15 cm (6 in.) 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 non-steroidal anti-inflammatory drugs (NSAIDs), such as ASA 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.

Medicine safety

Several types of medicines 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:

  • Antidepressants
  • Antihistamines
  • Bladder medicines that are not antibiotics
  • Blood pressure medicines
  • Decongestants
  • Diuretics
  • Heart medicines
  • Muscle spasm 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.2

  • Acupuncture. Some studies suggest that acupuncture might offer some improvement in dry eyes and dry mouth symptoms.
  • 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

Arthritis Society of Canada
393 University Avenue
Suite 1700
Toronto, ON  M5G 1E6
Phone: (416) 979-7228
Fax: (416) 979-8366
Email: info@arthritis.ca
Web Address: http://www.arthritis.ca/
 

The Arthritis Society provides funding for arthritis research and offers information on patient care, public education, and self-management of arthritis.


Canadian Ophthalmological Society
610-1525 Carling Avenue
Ottawa, ON  K1Z 8R9
Email: cos@eyesite.ca
Web Address: www.eyesite.ca/english/index.htm
 

The Canadian Ophthalmological Society is an association of eye doctors dedicated to helping the public take good care of their eyes and vision. This group provides educational information on eye conditions and diseases and eye safety.


Canadian Rheumatology Association
912 Tegal Place
Newmarket, ON  L3X 1L3
Phone: (905) 952-0698
Fax: (905) 952-0708
Email: cra@rogers.com
Web Address: http://www.rheum.ca
 

The Canadian Rheumatology Association is a professional organization of rheumatologists that promotes arthritis care and research in Canada through leadership, education, and communication.


References

Citations

  1. 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.
  2. Fox RI, et al. (2000). Update in Sjögren syndrome. Current Opinion in Rheumatology, 12(5): 391–398.

Other Works Consulted

  • Fife RS, et al. (2002). Cevimeline for the treatment of xerostomia in patients with Sjögren's syndrome. Archives of Internal Medicine, 162: 1293–1300.

Credits

By Healthwise Staff
Primary Medical Reviewer Anne C. Poinier, MD - Internal Medicine
Primary Medical Reviewer Andrew Swan, MD, CCFP, FCFP - Family Medicine
Specialist Medical Reviewer Stanford M. Shoor, MD - Rheumatology
Last Revised June 23, 2010

This information does not replace the advice of a doctor. Healthwise, Incorporated disclaims any warranty or liability for your use of this information.