SJOGREN'S SYNDROME

General Information

DEFINITION--The second most common autoimmune, rheumatic disorder (after rheumatoid arthritis). It may be a primary disorder or be associated with other connective tissue disorders (rheumatoid arthritis, scleroderma, systemic lupus erythematous, polymyositis).

BODY PARTS INVOLVED--May involve only the exocrine (mucus secreting) glands or involve other organs, such as the lung or kidneys.

SEX OR AGE MOST AFFECTED--Average age of occurrence is 50 and 90% of patients are female.

SIGNS & SYMPTOMS

• Dryness of the eyes that can cause foreign body sensation, gritty feeling, redness, burning, sensitivity to light, itching, a sensation of a "film" across the vision field and eye discharge.
• Dryness of the mouth that can cause difficulty in swallowing and talking, abnormal taste or smell, thirst, ulcers, dental cavities.
• Dryness of the vagina that can cause painful intercourse.
• Dryness of the upper respiratory tract that can cause nosebleeds, hoarseness, chronic nonproductive cough, ear infection, other respiratory infections.
• Parotid gland enlargement (sometimes referred to as "chipmunk face").
• Joint inflammation.
• Other symptoms include hair loss, generalized itching, fatigue, low-grade fever, muscle pain.

CAUSES--Unknown. Genetic, immunologic, hormonal and environmental factors may contribute to its development. Viral infection may trigger disorder in susceptible individual.

RISK INCREASES WITH

• Family history of autoimmune disorders.
• Rheumatoid arthritis.
• Scleroderma.
• Systemic lupus erythematosus.
• Polymyositis.

HOW TO PREVENT--No specific measures.

What To Expect

DIAGNOSTIC MEASURES--

• Medical history and exam by a doctor.
• Tests may include the Schirmer's test to measure quantity of tears produced in 5 minutes, other eye examinations, salivary flow studies, lip biopsy, and studies of the blood and urine.

APPROPRIATE HEALTH CARE

• Doctor's treatment.
• Treatment is directed to relieving the dryness of the eyes, mouth and other body parts.

POSSIBLE COMPLICATIONS

• Pulmonary infection.
• Increased disability.
• Renal failure (rare).
• Lymphoma (rare).

PROBABLE OUTCOME--Sjogren's syndrome is a chronic disorder and the prognosis is often related to an associated disorder. Treatment can relieve symptoms and help prevent complications.

How To Treat

GENERAL MEASURES--

• Meticulous oral hygiene is important. Regular dental visits should be scheduled.
• Wear sunglasses when outside to help protect eyes from dust, wind and strong light. Special moisture chamber spectacles may be helpful.
• Avoid rubbing the eyes.
• Soft contact lenses may be prescribed.
• Use a cool-mist, ultrasonic humidifier in the home. Clean humidifier daily.
• Avoid prolonged hot showers or baths.
• Warm compresses or heating pad may help ease joint pain or swollen gland discomfort.
• Avoid decongestants and antihistamines. They cause dry mouth.
• See Resources for Additional Information.

MEDICATION--Your doctor may prescribe:

• Artificial tears for eye dryness.
• Methylcellulose swab or spray for mouth dryness.
• Normal saline solution drops or aerosolized spray for respiratory dryness.
• K-Y Jelly as a lubricant for vaginal dryness.
• Corticosteroids and immunosuppressants for patients with severe symptoms.
• Nystatin for mouth infections.

ACTIVITY--No restrictions, but may be limited by symptoms.

DIET--

• Avoid sugar, which contributes to dental caries.
• To ease mouth dryness, chew sugarless gum or suck on sugarless candies.
• Drink plenty of fluids, especially at mealtime.
• If mouth soreness prevents eating regular foods, drink high-calorie, high-protein liquid supplements to prevent malnutrition.

Call Your Doctor If

Symptoms worsen or don't improve with treatment.