Sjögren's syndrome is a chronic (long-lasting) disorder that causes insufficient moisture production in some glands of the body, primarily in the eyes and mouth.
Sjögren's syndrome occurs when a person's normally protective immune system attacks her/his body and damages moisture-producing glands, including salivary (saliva-producing) glands and lacrimal (tear-producing) glands. The lungs, bowel, and other organs may rarely be affected.
Sjögren's syndrome is named after the Swedish eye doctor, Henrik Sjogren, who first described the condition.
Sjögren’s syndrome is characterized by dry eyes and mouth. In some patients, the parotid glands may become visibly enlarged.
Sjögren's syndrome occurs in two basic forms:
It is important to know that most cases of dry eyes and mouth are not due to Sjogrens syndrome.
More than one million people in the United States have been diagnosed with Sjögren's syndrome (about 0.5 to 1 percent of the population). More than 90 percent of people affected by Sjögren's syndrome are women. The disease can affect people of any race or age, but affects mostly middle-aged individuals.
Normally, the immune system (the body's defense system) protects the body from infection and foreign substances such as bacteria and viruses.
In autoimmune diseases such as Sjögren's syndrome, the immune system triggers an inflammatory response when there are no foreign substances to fight off. This inflammatory response causes the body's white blood cells to attack and damage its own moisture-producing glands.
The exact cause for the abnormal immune response in Sjögren's syndrome is unknown. There are four factors that may work together to cause the medical problems:
Certain people may have a genetic or inherited factor that makes them more likely to develop Sjögren's syndrome.
The use of certain medications such as tricyclic antidepressants and antihistamines commonly cause the symptoms of Sjögren's syndrome, without inflammation or damage. Radiation treatments to the head and neck and other autoimmune disorders can also cause severely dry eyes and mouth. Hepatitic C, sarcoidosis, and HIV infection can also cause these dry symptoms.
Sjögren's syndrome is mostly characterized by dry eyes and mouth. In some patients, the parotid glands may become visibly enlarged.
The main symptoms of Sjögren's syndrome are:
Less common features of Sjögren's syndrome are:
The diagnosis of Sjögren's syndrome is based on several factors, including:
Secondary Sjögren's syndrome is generally diagnosed when someone with an established autoimmune disease such as rheumatoid arthritis or systemic lupus erythematosus develops extreme dryness of the eyes and mouth. This diagnosis only rarely requires a lip biopsy.
There is no cure for Sjögren's syndrome, but it can be treated and managed. The goals of treatment are to decrease discomfort and reduce the harmful effects of dryness. Generally, physicians use medications to control symptoms (symptomatic treatment). The type of treatment will be tailored to each patient's symptoms and needs.
Good oral hygiene
Good mouth/dental care may prevent or reduce dental decays, infections, or tooth loss:
Increasing eye moisture
Dry eyes are mainly treated with the use of artificial tears. A wide variety of over-the-counter products is available. Artificial tears can be used regularly and more often in dry environmental conditions such as on airplanes, in air-conditioned buildings, and on windy days.
While artificial tears are helpful, they often do not last long enough. Thicker preparations (gel form) that last longer are available. These are often used at bedtime because they can sometimes cause blurry vision. Eye doctors can prescribe an eye drop called Restasis to treat more severe form of dry eyes.
A small procedure called punctal plugs, to slow the disappearance of tears, is another treatment option when artificial tears are not sufficient.
Medications that tend to reduce body fluids should be avoided.
Mild pain-relieving medications (analgesics), including acetaminophen (such as Tylenol®) or nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Motrin®) and naproxen (Aleve®), may reduce muscle or joint pain.
The anti-rheumatic drug hydroxychloroquine has been beneficial in some patients for decreasing pain and salivary gland swelling. This drug generally does not help with dry symptoms or fatigue, however.
For the rare patient with internal organ involvement, steroids and immunosuppressive medications may be used. These include medicines such as prednisone (a steroid) and, rarely, chemotherapy-type medications.
Balance of rest and exercise
Guided exercise programs can help patients overcome fatigue, maintain flexibility, and overcome joint and muscle pain. Good sleep hygiene is helpful for improving fatigue and body pain.
Sjögren's Syndrome Foundation, Inc.
10701 Parkridge Blvd, Suite 170
Reston, VA 20191
Toll Free: (800) 475-6473
Local call: (301) 530-4420
Fax: (301) 530-4415
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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or healthcare provider. Please consult your healthcare provider for advice about a specific medical condition. This document was last reviewed on: 01/21/2019