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by Kassir K

Sjogren Syndrome

(Primary Sjogren Syndrome; Secondary Sjogren Syndrome)

Definition

Sjogren 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 2 types:
  • Primary Sjogren syndrome—occurs alone
  • Secondary Sjogren syndrome—occurs with other rheumatic conditions such as rheumatoid arthritis , scleroderma , or lupus
Salivary Glands
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Causes

The causes of Sjogren are unknown. Contributing factors may include:
  • Viral infections
  • Environmental factors
  • Heredity
  • Hormones

Risk Factors

Women and people between the ages of 40 to 60 years old are at increased risk. Factors that increase your risk for Sjogren include:

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
  • Lip biopsy
  • Urine tests—to check the protein levels in your urine
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:

Treatment

There is no cure for Sjogren syndrome. 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 . Your doctor will need to monitor you for this.

Prevention

There are no guidelines for preventing Sjogren 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
https://www.canada.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 syndrome. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T116074/Sjogren-syndrome . Updated August 15, 2017. Accessed December 21, 2017.
Venables PJ. Management of patients presenting with Sjogren's syndrome. Best Pract Res Clin Rheumatol. 2006;20:791-807.
7/7/2014 DynaMed Plus Systematic Literature Surveillance http://www.dynamed.com/topics/dmp~AN~T116074/Sjogren-syndrome : 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.
11/9/2015 DynaMed Plus Systematic Literature Surveillance http://www.dynamed.com/topics/dmp~AN~T116074/Sjogren-syndrome : Kuo CF, Grainge MJ, Valdes AM, et al. Familial aggregation of systemic lupus erythematosus and coaggregation of autoimmune diseases in affected families. JAMA Intern Med. 2015;175(9):1518-1526.
2/22/2017 DynaMed Plus Systematic Literature Surveillance http://www.dynamed.com/topics/dmp~AN~T116074/Sjogren-syndrome : Luciano N, Baldini, Tarantini G, et al. Ultrasonography of major salivary glands: a highly specific tool for distinguishing primary Sjögren's syndrome from undifferentiated connective tissue diseases. Rheumatology (Oxford). 2015;54(12):2198-2204.
8/1/2019EBSCO DynaMed Systematic Literature Surveillance http://www.dynamed.com/topics/dmp~AN~T116074/Sjogren-syndrome : Singh JA, Cleveland JD. The risk of Sjogren's syndrome in the older adults with gout: A medicare claims study. Joint Bone Spine. 2019 Feb 7 [Epub ahead of print].

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