Sjogren's Syndrome

 

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Original Editors - Cassie Shay from Bellarmine University's Pathophysiology of Complex Patient Problems project.

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Definition/Description

Sjogren's syndrome was first described by physician Henrik Sjogren in the early nineteen hundreds to explain the signs and symptoms of a group of women exhibiting chronic arthritis along with extremely dry eyes and dry mouth¹. It is now understood that Sjogren's syndrome is an autoimmune connective tissue disease in which the body's own immune system attacks moisture producing glands, causing inflammation in addition to reduction in both the quality and quantity of the glands' secretions^1,2,3,4,5. As observed by Dr. Sjogren, the glands responsible for producing saliva and tears, the salivary and lachrymal glands respectively, are the organs most notably impacted by the disease². However, Sjogren's syndrome is a systemic disorder in which many organs may be affected, including kidneys, gastrointestinal tract, blood vessels, respiratory tracts, liver, pancreas, and central nervous system³. Additionally, it is considered a rheumatic disease, like rheumatoid arthritis or lupus, and like these disease Sjogren's syndrome can cause inflammation in joints, muscles, skin, and other organs².

Prevalence

Sjogren's syndrome is the second most prevalent autoimmune rheumatic disease. In 2008, it was estimated that 1.3 million Americans were affected^2,3. Statistics from rheumotology clinics indicate that Sjogren's syndrome affects 0.5 - 1% of the general population, and approximately the same number of people have been diagnosed with Sjogren's syndrome and Systemic Lupus Erythematosus ⁴.

Although, it can affect individuals of all genders and ages, including children, symptoms usually occur in women between the ages of 45 and 55 years old¹. In fact, it is estimated that nine times as many women are affected when compared to men³. Among individuals with rheumatoid arthritis or systemic sclerosis, thirty percent have histological evidence of Sjogren's syndrome³.

Characteristics/Clinical Presentation

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Associated Co-morbidities

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Medications

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Diagnostic Tests/Lab Tests/Lab Values 

Diagnosis of this disease is based on a medical history, a physical exam, and results from some clinical and laboratory tests^1,2,3. Due to the nature of disease in that the symptoms are similar to other diseases and they appear gradually over time, it may take years for Sjogren's to be properly diagnosed². Nonetheless, diagnostic testing may include the following:

• The Schirmer's test assesses the function of the lacrimal glands by measuring the amount of tears they are producing. This test involves putting a strip of paper under the lower eyelid, waiting five minutes, then measuring how much moisture was absorbed by the paper. An individual who tests positive for Sjogren's syndrome will typically produce too few tears to get more than 8 millimeters of the paper wet².
• During a slit lamp examination an ophthalmologist magnifies and examines the eye for severe dryness and inflammation. This test may be perfomed in conjunction with a vital dye applied to the eyes to determing the extent to which the eyes may have been damaged secondary to the dryness².
• A mouth examination may be performed to determine if signs of mouth dryness are present. These signs may include, sticky oral mucosa, tooth decay and cavaties in certain locations, thick saliva, and redness inside the oral cavity. The physician may also be able to note inflamed or underproducing salivary glands during this examination².

Etiology/Causes

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Systemic Involvement

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Medical Management (current best evidence)

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Physical Therapy Management (current best evidence)

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Alternative/Holistic Management (current best evidence)

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Differential Diagnosis

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Case Reports/ Case Studies

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Resources

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Recent Related Research (from Pubmed)

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References

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