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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:

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

Factors that increase your risk for Sjogren's include:

  • Sex: female
  • Age: 40-60 years old
  • 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

The doctor will ask about your symptoms and medical history. A physical exam will be done. You may be referred to a specialist.

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
  • Slit-lap 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

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 unscented moisturizers to help relieve dry skin.

This condition is generally benign. However, people with severe cases are at increased risk for developing non-Hodgkin lymphoma. 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 August 15, 2013.

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 August 15, 2013.



Last reviewed August 2013 by Rosalyn Carson-DeWitt, MD; Michael Woods, 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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