Sjogren Syndrome

Sjogren Syndrome

Definition

Sjögren syndrome is an autoimmune disorder that causes a major decrease in lubricating fluids, such as tears and saliva. An autoimmune disorder is one in which the person's body attacks its own tissues for unknown reasons.

What is going on in the body?

In Sjögren syndrome, the body's natural defense mechanisms invade and destroy the moisture-producing glands in the body. These glands produce saliva, tears, and other lubricating fluids. Since the body produces less saliva and fewer tears, the eyes and mouth become dry. A person may have Sjögren syndrome alone or with other autoimmune disorders. These include rheumatoid arthritis and systemic lupus erythematosus.

Risks

What are the causes and risks of the disease?

Sjögren syndrome occurs in about 2 out of 10,000 people. People with Sjögren syndrome produce antibodies against the moisture-producing tissues in their own bodies for some unknown reason. Sjögren syndrome occurs in more women than men at a ratio of 9:1. It affects mostly middle-aged women but canoccur in all ages. Thirty percent of people who have other autoimmune disorders also suffer from secondary Sjögren syndrome.
New research findings suggest that autoimmune disorders may be triggered by a transfer of cells between the fetus and the mother during pregnancy. The study involved women with scleroderma, an autoimmune disorder involving the skin. These women have more fetal cells in their blood decades after a pregnancy than women who don't have scleroderma. While further research is needed to substantiate these findings, the study does offer an explanation for the much higher incidence of autoimmune disorders in women than in men.

Prevention

What can be done to prevent the disease?

There is no known prevention for Sjögren syndrome.

Diagnosed

How is the disease diagnosed?

The diagnosis of Sjögren syndrome begins with a history and physical exam. A series of blood tests may be ordered. Special tests may be done to check saliva production. Eyes are checked for corneal injuries, or scratches. The eyes arealso checked for their ability to produce tears. Sometimes a small sample of a gland that produces saliva is removed and checked under a microscope.

Long Term Effects

What are the long-term effects of the disease?

The course of Sjögren syndrome is highly unpredictable. It may get worse, get better, or stay the same. The most common long-term effects are due to the loss of lubricating fluids of the eyes and mouth. A person will need to prevent injuries to the eyes and mouth.

Other Risks

What are the risks to others?

Sjögren syndrome is not contagious and causes no risks to others.

Treatments

What are the treatments for the disease?

A person with Sjögren syndrome usually uses artificial tears to keep the eyes moist. Artificial saliva is used to prevent dry mouth. Pilocarpine (i.e., Salagen) and cevimeline (i.e., Evoxac) are available by prescription to help dry mouth. Frequent sips of water throughout the day may also be helpful.
Other helpful products include unscented skin lotion, saline nasal spray, and vaginal lubricants.
An individual with Sjögren syndrome should avoid medications that are known to dry up secretions. These medications include antihistamines, decongestants, and some sleeping medications.
Any other autoimmune disorder present will also need treatment. Medications are sometimes used to reduce the effects of the immune system on body tissues. These medications may include immunosuppressive medications, nonsteroidal anti-inflammatory drugs, or NSAIDs, and corticosteroids such as prednisone.

Side Effects

What are the side effects of the treatments?

The artificial tears and saliva have no side effects. Medications that suppress the body's immune system can leave a person more likely to get infections.

After Treatment

What happens after treatment for the disease?

Treatment of Sjögren syndrome is lifelong. Complications may include increased dental cavities, kidney failure, and sores or scratches on the eye that can lead to blindness.

Monitor

How is the disease monitored?

Any new or worsening symptoms should be reported to the healthcare provider.

Sources

Black JM. Matassarin-Jacobs E.: Medical-surgical nursing: clinical management for continuity of care. Ed 5; p 678-9. Philadelphia, 1997, W.B. Saunders.

Isselbacher KJ, Braunwald E, Wilson, JD, et al: Harrison's principles of internal medicine. ed 14; p 1901-1904. New York, 1998, McGraw-Hill.

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