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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:
- 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.
RESOURCES
American Autoimmune Related Diseases Association
http://www.aarda.org
http://www.aarda.org
Sjogren's Syndrome Foundation
http://www.sjogrens.org
http://www.sjogrens.org
CANADIAN RESOURCES
Alberta Health
http://www.health.alberta.ca
http://www.health.alberta.ca
Health Canada
https://www.canada.ca
https://www.canada.ca
References
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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.
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Ramos-Casals M, Tzioufas AG, Font J. Primary Sjögren's syndrome: new clinical and therapeutic concepts.
Ann Rheum Dis.
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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.
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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].
