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Sjogren's syndrome

Sjögren's syndrome is an autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva. It is named after Swedish ophthalmologist Henrik Sjögren (1899-1986), who first described it. Sjögren's syndrome is also associated with rheumatic disorders such as rheumatoid arthritis, and it is rheumatoid factor positive in 90 percent of cases. The hallmark symptoms of the disorder are dry mouth and dry eyes. more...

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In addition, Sjögren's syndrome may cause skin, nose, and vaginal dryness, and may affect other organs of the body, including the kidneys, blood vessels, lungs, liver, pancreas, and brain. Nine out of ten Sjögren's patients are women and the average age of onset is late 40s, although Sjögren's occurs in all age groups in both women and men. It is estimated to strike as many as 4 million people in the United States alone making it the second most common autoimmune rheumatic disease.

Diagnosis

Diagnosing Sjögren’s syndrome is complicated by the range of symptoms a patient may manifest, and the similarity between symptoms from Sjögren's syndrome and those caused by other conditions. Nevertheless, several tests can confirm a diagnosis of Sjögren's syndrome.

Blood tests can be done to determine if a patient has high levels of antibodies that are indicative of the condition, such as anti-nuclear antibody (ANA) and rheumatoid factor, which are associated with autoimmune diseases. Typical Sjögren syndrome ANA patterns are SSA/Ro and SSB/La, of which SSB/La is far more specific; SSA/Ro is associcated with numerous other autoimmune conditions but are often present in Sjögren's (Franceschini & Cavazzana I 2005).

The Schirmer test measures the production of tears: a strip of filter paper is held inside the lower eyelid for five minutes, and its wetness is then measured with a ruler. A slit-lamp examination is done to look for dryness on the surface of the eye. Salivary gland function can be tested by collecting saliva and determining the amount produced. A lip biopsy can reveal lymphocytes clustered around salivary glands, and damage to these glands due to inflammation.

Treatment

There is neither a known cure for Sjögren's syndrome nor a specific treatment to permanently restore gland secretion. Instead, treatment is generally symptomatic and supportive. Moisture replacement therapies such as artificial tears may ease the symptoms of dry eyes (some patients with more severe problems use goggles to increase local humidity or have punctal plugs inserted to help retain tears on the occular surface for a longer time). Additionally, Cyclosporine (Restasis®) is available by prescription to help treat chronic dry eye by suppressing the inflammation that disrupts tear secretion. Prescription drugs are also available that help to stimulate salivary flow, such as cevimeline (Evoxac™) and pilocarpine (Salagen™). Nonsteroidal anti-inflammatory drugs may be used to treat musculoskeletal symptoms. For individuals with severe complications, corticosteroids or immunosuppressive drugs may be prescribed. Also, disease-modifying antirheumatic drugs (DMARDs) such as methotrexate may be helpful.

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Sjögren's syndrome
From Gale Encyclopedia of Alternative Medicine, 4/6/01 by Kim Sharp

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 45-55 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. 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 a 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 lying 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 secretion 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.

Key Terms

Autoimmune disorder
A disorder in which the body's immune cells mistake the body's own tissues as foreign invaders; the immune cells then work to destroy tissues in the body.
Cornea
A transparent structure of the eye over the iris and pupil; light must pass through the cornea to make vision possible.
Immune system
The complex network of organs and blood cells that protect the body from foreign invaders, like bacteria, viruses, and fungi.

Further Reading

For Your Information

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

  • 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). P.O. Box 8923, New Fairfield, CT 06812-2510. (203) 746-6518 (800) 999-6673.
  • National Sjögren's Syndrome Association. 5815 N. Black Canyon Highway, #103, Phoenix, AZ 85015-2200. (602) 443-9844.

Gale Encyclopedia of Alternative Medicine. Gale Group, 2001.

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