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

Patient's with Sjogren's syndrome can present with a number of complaints. However, the most common symptoms of this autoimmunue disease are dry mouth and dry eyes. Individuals with this condition may describe their symptoms as they relate to their eyes as an irritation, burning, or gritty feeling, as though they have sand in their eyes^1,3. The dryness in their mouth may make it difficult to speak, swallow,chew, or it may make foods taste differently³.

While these are the hallmark signs of Sjogren's syndrome, a patient may present with a number of other complaints including joint and muscle pain, fatigue, skin rashes or extremely dry skin, weakness, numbness, digestive problems, and Raynauld's phenomenon^1,2. Females diagnosed with Sjogren's may also experience vaginal dryness and pain with intercourse².

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:

File:Lip Biopsy.jpg

Histopathologic image of a lip biopsy demonstrating lymphoid infiltration in a minor salivary gland. H&E stain.

• 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.
• A lip biopsy is a method used to determine if the dry mouth experienced by the patient is a symptom of Sjogren's syndrome. During this test, minor salivary glands are removed from the bottom lip and placed under a microscope. If the glands contain a certain arrangement of white blood cells, the test is positive for the salivary component of Sjogren's syndrome. 

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)

Individuals with Sjogren's syndrome have reduced physical capacity and tend to fatigue very easily. While research on the effects of exercise on individuals diagnosed with Sjogren's is limited, the available studies suggest these patients benefit from moderateto high-intensity levels of exercise³. This type of activity has a positive influence on aerobic capacity, fatigue, physical function, and mood.

Alternative/Holistic Management (current best evidence)

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Differential Diagnosis

There are several diseases that present with symptoms similar to Sjogren's syndrome, including the dry eyes and dry mouth³. Some differential diagnoses include:

• Systemic Lupus Erythematosus
  
• Vasculitis
• Thyroid disease
• Scleroderma
• Medication side-effects

Case Reports/ Case Studies

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Szturmowicz M, Wilinska E, Paczek A, et al. Primary Sjogren's syndrome with two extraglandular sites involvement-case report. Pneumonol. Alergol. Pol. 2010:78;445-450.

Resources

National Institute of Arthritis and Musculoskeletal and Skin Disease

American College of Rheumatology

Sjogren's Syndrome Foundation

Recent Related Research (from Pubmed)

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References

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