Sjögren's Syndrome

Sjögren's syndrome is a disorder in which the mouth and eyes become extremely dry. Sjögren's syndrome is often associated with other autoimmunedisorders (a condition in which the body's immune system mistakenly begins treating parts of the body as foreign invaders). While the immune cells shouldattack and kill invaders like bacteria, viruses, and fungi, these cells should not attack the body itself.

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.

When the disorder is limited to involvement of the eyes, with no other organor tissue involvement evident, it is called sicca complex.

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

The cause of Sjögren's syndrome has not been clearly defined, but several causes are suspected. The syndrome sometimes runs in families. Other potential causes include hormonal factors (since there are more women than men withthe 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.

The main problem in Sjögren's syndrome is dryness. The salivary glands are often attacked and slowly destroyed, leaving the mouth extremely dry and sticky feeling. Swallowing and talking become difficult. Normally, the salivawashes the teeth clean. Saliva cannot perform this function in Sjögren'ssyndrome, so the teeth develop many cavities and decay quickly. The parotidglands produce the majority of the mouth's saliva. They 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 glands of lacrimation) 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 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 important for digestion. The kidney may malfunction. About 33% of all patients with Sjögren's syndrome have othersymptoms unrelated to gland destruction. These symptoms include fatigue, decreased energy, fevers, muscle aches and pains, and joint pain.

A patient with Sjögren's syndrome must have at least three consecutive months of bothersome eye and/or mouth dryness. A variety of tests can 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 ofglands.

There is no cure for Sjögren's syndrome. Instead, treatment is aimed ateasing discomfort and complications associated with dryness. Artificial tearsmay need to be used up to every 30 minutes in order to avoid complications.Dry mouth is treated by sipping fluids slowly but constantly throughout the day. Sugarless chewing gum also can be helpful. An artificial saliva is available for use as a mouthwash. Careful dental hygiene is important in order to avoid tooth decay. Vaginal dryness can be treated with gel preparations. Steroid medications may be required when other symptoms of autoimmune disorders complicate Sjögren's syndrome, but they carry risks of cornea damage.

The prognosis for patients with primary Sjögren's syndrome is good. Although the condition is 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.

No one knows how to prevent this syndrome.

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