Sjogren syndrome

Definition

Sjogren syndrome is an autoimmune disorder in which the glands that produce tears and saliva are destroyed, causing dry mouth and dry eyes. However, the condition may affect many different parts of the body, including the kidneys and lungs.

Alternative Names

Xerostomia-Sjogren syndrome; Keratoconjunctivitis sicca - Sjogren; Sicca syndrome

Causes

The cause of Sjogren syndrome is unknown. It is an autoimmune disorder, which means the body mistakenly attacks healthy tissue. The syndrome occurs most often in women ages 40 - 50. It is rare in children.

Primary Sjogren syndrome is defined as dry eyes and dry mouth without another autoimmune disorder.

Secondary Sjogren syndrome occurs alone with another autoimmune disorder, such as:

• Polymyositis
• Rheumatoid arthritis
• Scleroderma
• Systemic lupus erythematosus

Sjogren syndrome affects about 1 - 4 million people in the United States.

Symptoms

Dryness of the mouth and eyes are the most common symptoms of this syndrome.

Eye symptoms:

• Itching eyes
• Feeling that something is in the eye

Mouth and throat symptoms:

• Difficulty swallowing or eating
• Loss of sense of taste
• Difficulty speaking
• Thick or stringy saliva
• Mouth sores or pain
• Hoarseness

Other symptoms may include:

• Fatigue
• Fever
• Change in the color of hands or feet
• Joint pain or joint swelling
• Swollen glands

Exams and Tests

A physical examination reveals dry eyes and mouth. There may be mouth sores because of the mouth dryness.

Tests:

• Schirmer's test of tear production
• Antinuclear antibodies (ANA) test
• Salivary gland biopsy
• Rheumatoid factor (possible)
• Tear test
• Slit lamp examination of the eyes

Treatment

The goal is to relieve symptoms.

• Dry eyes may be treated with artificial tears, eye-lubricating ointments, or cyclosoporine liquid.
• Tiny plugs can be placed in the tear drainage ducts to help the tears stay on the surface of the eye.

Disease modifying drugs (DMARDs) similar to those used for rheumatoid arthritis may improve the symptoms of Sjogren syndrome. These include tumor necrosis factor (TNF) inhibiting drugs.

Sipping water throughout the day and chewing sugarless gum may help relieve mouth dryness. Avoid medicines that can cause mouth dryness such as antihistamines and decongestants. Avoid alcohol.

Talk with your dentist about:

• Solutions to replace minerals in your teeth
• Saliva substitutes
• Drugs that help your salivary glands make more saliva

Frequent brushing and flossing of the teeth, and regular dental visits may prevent severe dental cavities caused by dry mouth.

Arthritis symptoms are commonly treated with nonsteroidal anti-inflammatory drugs (NSAIDs) and physical therapy.

Outlook (Prognosis)

The disease is usually not life-threatening. The outcome depends on what other diseases you have.

There is an increased risk of lymphoma.

Possible Complications

• Kidney failure (rare)
• Lymphoma
• Pulmonary infection
• Vasculitis (rare)

When to Contact a Medical Professional

Call for an appointment with your health care provider if you develop symptoms of Sjogren syndrome.

References

Kruszka P, O'Brian RJ. Diagnosis and management of Sjogren syndrome. Am Fam Physician. 2009;79:465-470.

Wu AJ. Optimizing dry mouth treatment for individuals with Sjögren's syndrome. Rheum Dis Clin North Am. 2008 Nov;34(4):1001-10, x.

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