by Carson-DeWitt R

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

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%5FAnd%5FConditions/Sj%C3%B6gren%5Fs%5FSyndrome. 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.

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