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Sjogren's Syndrome

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

    Sjogren's syndrome is an inflammatory disease. The immune system destroys cells in exocrine glands. It occurs most often in the tear and salivary glands. It is a lifelong condition. There are two types:
    • Primary Sjogren's syndrome—occurs alone
    • Secondary Sjogren's syndrome—occurs with other rheumatic conditions such as rheumatoid arthritis, scleroderma, or lupus
    Salivary Glands
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  • Causes

    The causes of Sjogren's are unknown. Contributing factors may include:
    • Viral infections
    • Environmental factors
    • Heredity
    • Hormones
  • Risk Factors

    Women and people between the ages of 40-60 years old are at increased risk. Factors that increase your risk for Sjogren's include:
    • Other rheumatic or autoimmune diseases
    • Certain gene markers
  • Symptoms

    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
    • Dental problems
    • Swollen salivary glands
    • Vaginal dryness
    • Skin rashes
    • Joint pain, swelling, and stiffness
    • 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
  • Diagnosis

    You will be asked about your symptoms and medical history. A physical exam will be done. You may be referred to a specialist. You may also be referred to a dentist for an exam.
    Your bodily fluids and tissues may be tested. This can be done with:
    • Blood tests
    • Urine tests
    • Lip biopsy
    Your eyes may be tested. This can be done with:
    • Schirmer test to measure tear production
    • Slit-lamp examination
    Images may also be taken of your bodily structures. This can be done with a chest x-ray.
  • Treatment

    There is no cure for Sjogren's. No treatment can restore the ability of the glands to produce moisture. The goal of treatment is to relieve symptoms.
    Treatments include:
    Medication
    You may be given medications to relieve:
    • Dryness
    • Joint and muscle pain
    • Inflammation and swelling
    Lifestyle Measures
    Lifestyle changes may help to relieve symptoms. These include:
    • Exercise to relieve stiffness in the joints
    • Sipping liquids and sucking on sugar-free candies to relieve dryness
    • Good oral hygiene and regular dental checkups
    • Using unscented moisturizers to help relieve dry skin
    People with severe cases of this syndrome are at increased risk for developing cancers such as non-Hodgkin lymphoma and thyroid cancer. This is a cancer of the white blood cells. Your doctor will need to monitor you for this.
  • Prevention

    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 http://www.health.alberta.ca

    Health Canada http://www.hc-sc.gc.ca

    References

    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.

    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, 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. American College of Rheumatology website. Available at: http://www.rheumatology.org/Practice/Clinical/Patients/Diseases%5FAnd%5FConditions/Sj%C3%B6gren%5Fs%5FSyndrome. Updated July 2012. Accessed December 16, 2014.

    Venables PJ. Management of patients presenting with Sjogren's syndrome. Best Pract Res Clin Rheumatol. 2006;20:791-807.

    7/7/2014 DynaMed's Systematic Literature Surveillance http://www.ebscohost.com/dynamed: Liang Y, Yang Z, et al. Primary Sjogren's syndrome and malignancy risk: a systematic review and meta-analysis. Ann Rheum Dis. 2014 Jun;73(6):1151-1156.

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