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Sjogren's Syndrome

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 destroys moisture-producing glands, including salivary (saliva-producing) glands and lacrimal (tear-producing) glands. The lungs, bowel, and other organs may be affected, but relatively less often.

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.

What are the symptoms of Sjögren's syndrome?

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:

  • Extremely dry eyes, causing a feeling of grit or sand, burning, and redness
  • Inner angle thick secretions
  • Extremely dry mouth and throat, causing:
    • difficulty chewing and swallowing, especially dry food such as crackers
    • decreased or altered sense of taste
    • difficulty speaking
    • increase in dental cavities and even tooth loss at early age
    • dry cough or hoarseness
  • Enlarged parotid glands (located at the angle of jaw), looking like an infection
  • Excessive fatigue
  • Aches and pains in muscles and joints, and even the whole body, similar to fibromyalgia pain

Less common features of Sjögren's syndrome are:

  • Irritation of the nerves in the arms, hands, legs, or feet (neuropathy)
  • Feeling of numbness or tingling
  • Thyroid gland abnormalities
  • Skin rashes
  • Memory loss, difficulty concentrating or confusion
  • Gastrointestinal problems, such as acid reflex, bloating, abdominal pain, or diarrhea
  • Inflammation of the lungs, kidneys (unlike lupus nephritis), liver, or pancreas
  • Cancer of the lymphatic tissue (occurs in up to 5% of patients with the disease)

What causes Sjögren's syndrome?

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 destroy 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:

  • abnormal immune response
  • sex hormones
  • inheritance, and
  • environment.

Certain people may have a genetic or inherited factor that makes them more likely to develop Sjögren's syndrome.

What are the forms of Sjögren's syndrome?

Sjögren's syndrome occurs in two basic forms:

  • Primary Sjögren's syndrome – the disease by itself, not associated with any other illness
  • Secondary Sjögren's syndrome – disease that develops in the presence of another autoimmune disease such as rheumatoid arthritis, systemic lupus erythematosus, or psoriatic arthritis

Who is affected by Sjögren's syndrome?

More than one million people in the United States have Sjögren's syndrome (about 0.5 to 1% of the population). More than 90% 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.

How is primary Sjögren's syndrome diagnosed?

The diagnosis of Sjögren's syndrome is based on several factors, including:

  • Presence of dry eyes and mouth. Dry eyes can be diagnosed by an ophthalmologist (eye doctor) by measuring tear production or carefully examining the cornea (clear part of the eye).
  • Certain laboratory tests also suggest that dry eyes and mouth are caused by autoimmune mechanisms. Examples include the presence of autoantibodies in the blood, known as ANA, anti-SSA, or anti-SSB (also known as anti-Ro or anti-La), and even rheumatoid factor.
  • Biopsy of the inner lip (performed in some cases to prove the diagnosis of primary Sjögren's syndrome). The biopsy may show the inflammation that is damaging the salivary glands.

How is secondary Sjögren's syndrome diagnosed?

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.

Can other problems mimic Sjögren's syndrome?

The use of certain medications such as tricyclic antidepressants and antihistamines can cause the symptoms of Sjögren's syndrome. 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.

How is Sjögren's syndrome treated?

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:

  • Toothpastes (biotene type) and oral gels are available for people with dry mouth symptoms. These products may also have antibacterial action to reduce the severity of dental cavities over a long period of time.
  • Chewing sugar-free gums can be helpful.
  • Taking frequent sips of water without swallowing (spitting it out) may improve dry mouth.
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

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 Motrin® and Aleve®), can reduce muscle or joint pain.

In some patients, the anti-rheumatic drug hydroxychloroquine has been beneficial in decreasing pain and salivary gland swelling and improving fatigue, muscle pain, joint pain, or rash. This drug generally does not help with dry symptoms, however.

For patients with internal organ symptoms (particularly when the disease affects internal organs), 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.

How can I learn more about Sjögren's Syndrome?

Sjögren's Syndrome Foundation, Inc.
6707 Democracy Blvd Ste 325
Bethesda, MD 20817
1.800.4.SJOGREN
Fax: 301.530.4415
www.sjogrens.org

References

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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 health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 11/3/2014…#4929

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This information is provided by Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition.

© Copyright 2014 Cleveland Clinic. All rights reserved.