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    TOP

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

  • Viral infections
  • Environmental factors
  • Heredity
  • Hormones

Risk Factors    TOP

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    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
  • 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    TOP

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    TOP

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    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:

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:
...(Click grey area to select URL)
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
...(Click grey area to select URL)
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
Last Updated: 12/20/2014

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