Sjogren's Syndrome

(Primary Sjogren's Syndrome; Secondary Sjogren's Syndrome)

Pronounced: show-GRENS
by Editorial Staff and Contributors

Definition

Sjogren's syndrome is an inflammatory disease. The immune system destroys cells in exocrine glands. It occurs most often the tear and salivary glands. It is a lifelong condition. There are two types:

Salivary Glands

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Causes

The causes of Sjogren's are unknown. Contributing factors may include:

  • Viral infections
  • Heredity
  • Hormones

Risk Factors    TOP

Factors that increase your risk for Sjogren's include:

  • Sex: female
  • Age: 40-60 years old
  • Other rheumatic or autoimmune diseases

Symptoms    TOP

Symptoms may include:

  • Red, burning, itching, and/or dry eyes
  • Dry mouth
  • Difficulty swallowing
  • Loss of taste and smell
  • Dry skin, nose, throat, and/or lungs
  • Swollen salivary glands
  • Severe dental cavities caused by dry mouth
  • Oral yeast infections
  • Vaginal dryness
  • Skin rashes
  • Joint and muscle pain
  • Fatigue

In some cases, other parts of the body are affected as well. These include:

  • Blood vessels
  • The nervous system
  • Organs such as the lungs, liver, pancreas, kidneys, and thyroid

Anti Ro antibodies are often present with Sjogren's. These may cross to the baby during pregnancy. In some cases, they can cause neonatal lupus or infant heartblock.

Diagnosis    TOP

This disease can affect many areas on your body. You may have to see several specialist before a final diagnosis is made. Dry eyes and mouth also increase with age. They can also be caused by medicine. The doctor will ask about your symptoms and medical history. A physical exam will be done.

Tests may include:

  • Blood tests—including rheumatoid factor and other tests used to detect and monitor autoimmune diseases
  • Chest x-ray —a test that uses radiation to take a picture of structures inside the body, in this case the lungs
  • Lip biopsy —removal of a sample of lip tissue to look for inflammation in the accessory salivary glands located there
  • Schirmer test—placement of small pieces of paper between the lower eyelid and eyeball to see how much moisture is being produced
  • Slit-lamp examination—a detailed exam of the eye with an adjustable light
  • Urine test—to check kidney function

Treatment    TOP

There is no cure for Sjogren's. No treatment can restore the ability of the glands to produce moisture. Patients with Sjogren’s have an increased incidence of non-Hodgkin’s lymphoma. They should be screened aggressively. The goal of treatment is to relieve symptoms.

Treatments include:

Medication

To help relieve dryness:

  • Artificial tears, artificial saliva, and vaginal lubricants
  • Pilocarpine —ocular and oral dryness
  • Cevimeline —requires less frequent dosing than pilocarpine, may cause nausea

To relieve joint and muscle pain:

  • Aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs)

To relieve inflammation / swelling:

  • Plaquenil —antimalarial drug with anti-inflammatory properties
  • Steroids
  • Methotrexate —a steroid-sparing agent

Lifestyle Measures

  • Mild exercise can help relieve stiffness in the joints.
  • To help relieve dry mouth, sip liquids often and suck on sugar-free candies.
  • Brush, floss, and see your dentist regularly. This can help to prevent cavities.
  • Use nonscented moisturizers to help relieve dry skin.

This condition is generally benign. However, people with severe cases are at increased risk for developing lymphoma. This is a cancer of the white blood cells. Your doctor will need to monitor you for this.

Prevention    TOP

There are no guidelines for preventing Sjogren's syndrome. The cause is unknown.

RESOURCES:

American Autoimmune Related Diseases Association
http://www.aarda.org/
Sjogren's Syndrome Foundation
http://www.sjogrens.org/

CANADIAN RESOURCES:

Alberta Health and Wellness
http://www.health.alberta.ca/
Health Canada
http://www.hc-sc.gc.ca/

References:

Arthritis Foundation website. Available at: http://www.arthritis.org/ .
The Cleveland Clinic Foundation website. Available at: http://www.clevelandclinic.org/ .
Fox RI. Sjogren’s syndrome. Lancet. 2005;366:321-331.
Kassan SS, Montsopolous HM. Clinical manifestations of Sjogren’s disease. Arch Intern Med. 2004;164:1275-1284.
National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health (NIH) website. Available at: http://www.niams.nih.gov/ .
Papas, et al. Successful treatment of dry mouth and dry eye symptoms in Sjogren's syndrome patients with oral pilocarpine: a randomized, placebo-controlled, dose-adjustment study. J Clin Rheumatol . 2004;10:169-177.
Pertovaara M, Korpela M, Uusitalo H, et al. Clinical follow up study of 87 patients with sicca symptoms (dryness of eyes or mouth, or both). Ann Rheum Dis. 1999; 58:423.
Ramos-Casals M, Tzioufas AG, Font J. Primary Sjögren's syndrome: new clinical and therapeutic concepts. Ann Rheum Dis. 2005; 64:347.
Sjogren's Syndrome Foundation website. Available at: http://www.sjogrens.org .
University of Washington Orthopaedics and Sports Medicine website. Available at: http://www.orthop.washington.edu/ .
Venables PJ. Management of patients presenting with Sjogren's syndrome. Best Pract Res Clin Rheumatol . 2006;20:791-807.
Last reviewed December 2011 by Rosalyn Carson-DeWitt, MD
Last Updated: 12/30/2011
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