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

Pronounced: show-GRENS

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_And_Conditions/Sj%C3%B6gren_s_Syndrome. 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.



Last reviewed December 2014 by Kari Kassir, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.

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