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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:
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
  • Swollen salivary glands
  • Severe dental cavities caused by dry mouth
  • Oral yeast infections
  • 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 June 3, 2014.

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

What you need to know about Sjogren's syndrome. Cleveland Clinic website. Available at: http://www.clevelandclinic.org/health/health-info/docs/0200/0220.asp. Accessed June 3, 2014.

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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