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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

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:

Certain genetic markers
Family history of autoimmune diseases such as systemic lupus erythematosus
History of other autoimmune diseases
Gout

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:

Chest x-ray .
Ultrasound of the salivary glands

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].

Revision Information

Reviewer: Kari Kassir, MD
Review Date: 11/2018
Update Date: 08/02/2019

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