Sjögren's syndrome

More about SjöGren's Syndrome: Causes and symptoms Diagnosis Treatment Prognosis Prevention Resources

Definition

Sjögren's syndrome is a disorder where the mouth and eyes become extremely dry. Sjögren's syndrome is often associated with other autoimmune disorders.

Description

Like other autoimmune disorders, Sjögren's syndrome occurs when the body's immune system mistakenly begins treating parts of the body as foreign invaders. While the immune cells should attack and kill invaders like bacteria, viruses, and fungi, these cells should not attack the body itself. In autoimmune disorders, however, cells called antibodies see tissues of the body as foreign, and help to start a chain of events that results in damage and destruction of those tissues.

There are three types of Sjögren's syndrome. Primary Sjögren's syndrome occurs by itself, with no other associated disorders. Secondary Sjögren's syndrome occurs along with other autoimmune disorders, like systemic lupus erythematosus, rheumatoid arthritis, scleroderma, vasculitis, or polymyositis. When the disorder is limited to involvement of the eyes, with no other organ or tissue involvement evident, it is called sicca complex.

Women are about nine times more likely to suffer from Sjögren's syndrome than are men. It affects all age groups, although most patients are diagnosed when they are between 40 and 55 years old. Sjögren's syndrome is commonly associated with other autoimmune disorders. In fact, 30% of patients with certain autoimmune disorders will also have Sjögren's syndrome.

— Rosalyn Carson-DeWitt, MD

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n.
A chronic inflammation of the lachrymal and salivary glands, often accompanied by rheumatoid arthritis and the presence of autoantibodies in the blood, occurring chiefly among women.

[After Henrik Samuel Conrad Sjögren (1899-1986), Swedish ophthalmologist.]

Key Terms: Autoimmune disease, Coxsackievirus, Lymphocytes, Lymphoma.

Description

Sjögren's syndrome (SS) is an autoimmune disease, which means that the immune system has mounted an attack against specific tissues of the body. For example, most patients with Sjögren's syndrome carry antibodies to molecules found in the nucleus of cells (antinuclear antibodies). Although Sjögren's syndrome can affect practically any organ in the body, it is characterized by dry mouth (xerostomia) and dry eyes (xerophthalmia). These hallmark symptoms are known as "sicca symptoms." Sjögren's syndrome goes by many names which include Sjögren's disease, dry-mouth and dry-eyes disease, sicca complex, and sicca syndrome. The disorder is named for Henrik Sjögren, a Swedish ophthalmologist.

Symptoms of Sjögren's syndrome include dry mouth, difficulty or inability to swallow (dysphagia), tooth decay (dental caries), impaired taste and smell, dry eyes, eye pain, eye redness, muscle pain (myalgia), and fatigue. Many patients develop a variety of skin problems that include dry patches, vasculitis, and cutaneous B-cell lymphoma. Other less common symptoms include diarrhea, headaches, joint pain (arthralgia), muscle weakness, hair loss (alopecia), and dry cough. About 33% of patients develop arthritis. Patients with cancer of lymphoid tissue (lymphoma) and Sjögren's syndrome have fever, nerve involvement, low numbers of red blood cells (anemia) and white blood cells (lymphopenia), inflammation of blood vessels of the skin (skin vasculitis), and disease of the lymph nodes (lymphadenopathy) much more frequently than patients with Sjögren's syndrome alone.

The symptoms of Sjögren's syndrome can have a pronounced effect on quality of life. Besides causing discomfort, the symptoms also disrupt sleep, which can have side effects such as fatigue, difficulty concentrating, and depression. Patients with Sjögren's syndrome are at risk for tooth decay and yeast infections in the mouth (erythematous candidiasis). Approximately 5% of the patients with Sjögren's syndrome develop malignant lymphoma.

SS is found in all races and ethnic groups. It is thought to affect between 0.1% and 3% of the population in the United States; this range reflects the lack of a uniform set of diagnostic criteria. According to the American College of Rheumatology, between 1 million and 4 million Americans have Sjögren's syndrome.

Causes

The cause of Sjögren's syndrome is unknown, although several viruses are suspected triggers of the autoimmune reaction. In 2004 a team of researchers in Greece presented evidence that a coxsackievirus may be the cause of SS. The sicca symptoms of Sjögren's syndrome are caused by the invasion and multiplication of white blood cells (lymphocytes) into the salivary glands and tear glands. The lymphocytes destroy the gland tissue and cause the glands to malfunction, reducing the production of tears and saliva. This invasion by lymphocytes, however, does not fully account for the sicca symptoms. Other, as yet unidentified, factors play a role in the development of the sicca symptoms.

Sjögren's syndrome can occur in combination with certain cancers. For more than half of the patients with non-Hodgkin lymphoma, the lymphoma is located in the salivary glands, causing them to malfunction. Graft-vs.-host disease in patients who have undergone bone marrow transplantation can cause eye problems similar to those seen in Sjögren's syndrome. Both chemotherapy and radiation therapy to the head and neck can cause xerostomia.

Treatments

There is no cure for Sjögren's syndrome. Therefore, treatment is aimed at relieving symptoms. Dry eyes may be treated with eye drops and avoidance of drying conditions such as wind, hair dryers, and medications that cause dry eyes (e.g. tricyclic antidepressants). Eyeglasses may protect the eyes from wind. The lower tear ducts may be blocked with silicone plugs (punctal occlusion) to conserve natural tears. Use of humidifiers, both at home and at work, can significantly reduce sicca symptoms. Saliva substitutes and sugar-free hard candies or chewing gum, which stimulate salivation, can reduce sicca symptoms. The drugs pilocarpine (Salagen) and cevimeline (Evoxac) can increase salivation. Cevimeline also has been found effective in relieving the symptom of dry eye in SS as well. Pain may be relieved by nonsteroidal anti-inflammatory drugs (e.g. Aleve) or other pain medications.

The patient with Sjögren's syndrome should faithfully conduct routine daily oral hygiene consisting of tooth brushing two to three times, flossing once, and utilizing medicated rinses as prescribed by the physician. Fluoride varnishes applied by a dentist and nightly fluoride treatments can help to prevent dental caries. Brushing and flossing should be performed carefully to prevent damage to the weakened oral mucosa.

Alternative and Complementary Therapies

In a controlled clinical study, the herbal vitamin supplement Longo Vital was shown to increase the rate of salivation. Sicca symptoms may be reduced by acupuncture. Papayas contain papain, which is an enzyme that breaks up proteins. Eating papayas, drinking papaya juice, or drinking a solution of crushed papain tablets in water can liquefy thick saliva. Drinking a solution of meat tenderizer (which contains papain) in water is another alternative.

Resources

Books

Beers, Mark H., MD, and Robert Berkow, MD, editors. "Diffuse Connective Tissue Disease." Section 5, Chapter 50 In The Merck Manual of Diagnosis and Therapy. Whitehouse Station, NJ: Merck Research Laboratories, 2002.

Iwamoto, Ryan R. "Xerostomia." In Cancer Symptom Management, edited by Connie H. Yarbro, Margaret H. Frogge, and Michelle Goodman, 2nd ed. Sudbury, MA: Jones and Bartlett Publishers, 1999.

Periodicals

Bell, Mary, et al. "Sjögren's Syndrome: A Critical Review of Clinical Management." The Journal of Rheumatology 26, no. 9 (2001): 2051–2059.

Daniels, Troy E. "Evaluation, Differential Diagnosis, and Treatment of Xerostomia." Current Opinion in Rheumatology 27 (2001): 6–9.

Fox, Robert I., and Paul Michelson. "Approaches to the Treatment of Sjögren's Syndrome." Current Opinion in Rheumatology 27 (2000): 15–20.

Fox, Robert I. "Update in Sjögren Syndrome." Current Opinion in Rheumatology 12 (2000): 391–398.

Francis, Mark L., MD. "Sjogren Syndrome." eMedicine July 1, 2004. .

Ono, M., E. Takamura, K. Shinozaki, et al. "Therapeutic Effect of Cevimeline on Dry Eye in Patients with Sjögren's Syndrome: A Randomized, Double-Blind Clinical Study." American Journal of Ophthalmology 138 (July 2004): 6–17.

Roguedas, A. M., L. Misery, B. Sassolas, et al. "Cutaneous Manifestations of Primary Sjögren's Syndrome Are Underestimated." Clinical and Experimental Rheumatology 22 (September-October 2004): 632–636.

Stevenson, H. A., M. E. Jones, J. L. Rostron, et al. "UK Patients with Primary Sjögren's Syndrome Are at Increased Risk from Clinical Depression." Gerodontology 21 (September 2004): 141–145.

Triantafyllopoulou, A., N. Tapinos, and H. M. Moutsopoulos. "Evidence for Coxsackievirus Infection in Primary Sjögren's Syndrome." Arthritis and Rheumatism 50 (September 2004): 2897–2902.

Organizations

American College of Rheumatology. 1800 Century Place, Suite 250, Atlanta, GA 30345-4300. (404) 633-3777. Fax: (404) 633-1870. .

Sjögren's Syndrome Foundation, Inc. 8120 Woodmont Avenue, Bethesda, MD 20814. (800) 475-6473. Fax: (301) 718-0322..

Other

American College of Rheumatology Fact Sheet. "Sjögren's Syndrome." .

MGH Virtual Brain Tumor Center. [cited June 22, 2001]. .

—Belinda Rowland, Ph.D.; Rebecca J. Frey, PhD

n.pr
sicca syndrome, xerodermostecisis

Condition related to deficient secretion of salivary, sweat, lacrimal, and mucous glands (xerostomia, keratoconjunctivitis, rhinitis, dysphagia), increased size of salivary glands, and polyarthritis.

Definition

Sjögren's syndrome is an autoimmune disorder in which the mouth and eyes become extremely dry. Sjögren's syndrome is often associated with other autoimmune disorders.

Description

Like other autoimmune disorders, Sjögren's syndrome occurs when the body's immune system mistakenly considers parts of the body as foreign invaders. People with this disease have abnormal proteins in their blood, suggesting that their immune system is reacting against their own tissue. While the immune cells should attack and kill invaders like bacteria, viruses, and fungi, these cells should not attack the body itself. In autoimmune disorders, however, cells called antibodies see tissues of the body as foreign, and help to start a chain of events that results in damage and destruction of those tissues.

There are three types of Sjögren's syndrome. Primary Sjögren's syndrome occurs by itself with no other associated disorders. Secondary Sjögren's syndrome occurs along with other autoimmune disorders, like systemic lupus erythematosus, rheumatoid arthritis, scleroderma, vasculitis, or polymyositis. When the disorder is limited to involvement of the eyes, with no other organ or tissue involvement evident, it is called sicca complex.

Women are about 10 times more likely to suffer from Sjögren's syndrome than are men. It affects all age groups, although most patients are diagnosed when they are between 45 and 55 years old. Sjögren's syndrome is commonly associated with other autoimmune disorders. In fact, 30% of patients with certain autoimmune disorders will also have Sjögren's syndrome.

Causes & Symptoms

The cause of Sjögren's syndrome has not been clearly defined, but several causes are suspected. For instance, genetic factors play a role, in that the syndrome sometimes runs in families. In fact, autoimmune diseases in general tend to occur in families with certain genetic characteristics. In late 2001, researchers announced discovery of the genetic markers that predict increased risk of many autoimmune disorders like rheumatoid arthritis, multiple sclerosis, and lupus.

Other potential causes include hormonal factors (since there are more women than men with the disease) and viral factors. The viral theory suggests that the immune system is activated in response to a viral invader, but then fails to turn itself off. Some other immune malfunction then causes the overly active immune system to begin attacking the body's own tissues. Sjögren's syndrome is thought to be the end result of several factors including genetic, immunologic, hormonal, and possibly infectious.

The main problem in Sjögren's syndrome is dryness. The salivary glands and secretory glands (mucous/liquid) are often attacked and slowly destroyed, leaving the mouth extremely dry and sticky-feeling. Swallowing and talking become difficult. Normally, the saliva washes the teeth clean. Saliva cannot perform this function in Sjögren's syndrome, so the teeth develop many cavities and decay quickly. The parotid glands produce the majority of the mouth's saliva. These glands are located over the jaw bones, behind the area of the cheeks and in front of the ears, and may become significantly enlarged in Sjögren's syndrome.

The eyes also become extremely dry as the tear glands (called lacrimal glands) are slowly destroyed. Eye symptoms include itching, burning, redness, increased sensitivity to light, and thick secretions gathering at the eye corners closest to the nose. The cornea may have small irritated pits in its surface (ulcerations).

Destruction of secretary glands in other areas of the body may cause a variety of symptoms. In the nose, dryness may result in nosebleeds. In the rest of the respiratory tract, the rates of ear infection, hoarseness, bronchitis, and pneumonia may increase. Vaginal dryness can be quite uncomfortable. Rarely, the pancreas may slow production of enzymes critical for digestion. The kidney may malfunction. About 33% of all patients with Sjögren's syndrome have other symptoms unrelated to gland destruction. These symptoms include fatigue, decreased energy, fevers, muscle aches and pains, and joint pain.

Patients who also have other autoimmune diseases will suffer from the symptoms specific to those conditions. A rare but serious complication of Sjögren's syndrome is inflammation of the blood vessels (vasculitis), which can damage tissues supplied by these blood vessels.

Diagnosis

Diagnosis of Sjögren's syndrome is based on the patient having at least three consecutive months of bothersome eye and/or mouth dryness. A variety of tests can then be done to determine the quantity of tears produced, the quantity of saliva produced, and the presence or absence of antibodies that could be involved in the destruction of glands.

Treatment

There is no cure for Sjögren's syndrome. Instead, treatment usually attempts to reduce the discomfort and complications associated with dryness of the eyes and mouth (and other areas). Artificial tears are available, and may need to be used up to every 30 minutes. By using these types of products, the patient is more comfortable and avoids the complications associated with eyes that are overly dry. Dry mouth is treated by sipping fluids slowly but constantly throughout the day. Sugarless chewing gum can also be helpful. An artificial saliva is available for use as a mouthwash. Careful dental hygiene is important in order to avoid tooth decay, and it is wise for patients to decrease sugar intake.

Allopathic Treatment

Vaginal dryness can be treated with certain gel preparations. Steroid or immunosuppressive medications may be required when other symptoms of autoimmune disorders complicate Sjögren's syndrome. However, these medications should be avoided when possible because they may thin the cornea and make it even more susceptible to injury.

Expected Results

The prognosis for patients with primary Sjögren's syndrome is particularly good. Although the condition is quite annoying, serious complications rarely occur. The prognosis for patients with secondary Sjögren's syndrome varies, since it depends on the prognosis for the accompanying autoimmune disorder.

Prevention

Since the cause of Sjögren's syndrome is unknown, there are no known ways to prevent this syndrome.

Resources

Books

Aaseng, Nathan. Autoimmune Diseases. New York: F. Watts, 1995.

Koopman, D. Arthritis and Allied Conditions. New York: Williams and Wilkins, 1997.

Moutsopoulos, Haralampos M. "Sjögren's Syndrome." In Harrison's Principles of Internal Medicine, edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1998.

Talal, N., et al. Sjögren's syndrome: Clinical and Immunological Aspects. Berlin: Springer, 1987.

Periodicals

Moran, M. "Autoimmune Diseases Could Share Common Genetic Etiology." American Medical News. 44, no. 38: (October 8, 2001):38.

Moutsopoulos, H. M., and P. G. Vlachoyiannopoulos. "What Would I Do If I Had Sjögren's syndrome?" Rheumatology Review 2 (1993): 17+.

Moutsopoulos, H. M., and P. Youinou. "New Developments in Sjögren's syndrome." Current Opinion in Rheumatology 3 (1991): 815+.

Organizations

National Institute of Arthritis & Skin Diseases. Building 31, Room 4C05, Bethesda, MD 20892-2350. (301) 496-8188. http://nih.gov/niams.

National Organization for Rare Disorders (NORD). 55 Kenosia Avenue, Danbury, CT 06813 (203) 744-0100 (toll-free # is the same).

National Sjögren's Syndrome Association. 5815 N. Black Canyon Highway, #103, Phoenix, AZ 85015-2200. (602) 443-9844.

[Article by: Kim Sharp; Teresa Norris]

A symptom complex marked by keratoconjunctivitis sicca and xerostomia often occurring in association with other inflammatory disorders such as immune-mediated arthritis and systemic lupus erythematosus. Reported in dogs.