Sjögren’s Syndrome
Professional Refresher

Sjögren’s syndrome is a chronic autoimmune disorder that causes the white blood cells to attack the moisture-producing glands. The level of severity differs among individuals, and it can go undiagnosed for a long period of time in some people. It takes an average of 6.5 years from the onset of symptoms to diagnosis.

Sjögren’s syndrome can occur in association with other autoimmune diseases, including systemic lupus erythematosus, rheumatoid arthritis, scleroderma, systemic sclerosis, cryoglobulinemia, or polyarteritis nodosa. The disease can affect the eyes, mouth, parotid gland, pancreas, gastrointestinal system, blood vessels, lungs, kidneys, skin, and nervous system. This disease is usually not fatal, but 1% of patients may develop non-Hodgkin lymphoma. About 10% of patients may develop pseudolymphoma. The female-to-male ratio for this condition is 9:1, and most people are diagnosed in their 40s or 50s.

Signs and symptoms of Sjögren’s syndrome
Signs and symptoms include:

• Ocular dryness, leading to:

Chronic keratoconjunctivitis
Corneal ulcers

• Oral dryness, leading to:

Caries
Fissures
Difficulty speaking
Difficulty chewing
Sore or cracked tongue
Dry or burning throat
Dry or peeling lips
Change in taste or smell
Oral thrush
Persistent dry cough
Difficulty swallowing

• Musculoskeletal fatigue, arthralgias, and myalgias
• Dry skin
• Dry nose, recurrent sinusitis, nosebleeds
• Vaginal dryness
• Vasculitis
• Lymphadenopathy
• Polyneuropathies
• Renal involvement—glomerulonephritis and interstitial nephritis
• Parotid gland enlargement
• Pneumonia, bronchitis, and other lung conditions
• Hepatitis or cirrhosis
• Numbness, tingling, and burning in the hands and feet
• Digestive problems, gastroparesis, and autoimmune pancreatitis
• Heartburn  and reflux esophagitis
• Concentration or memory problems
• Hashimoto’s thyroiditis (commonly found in people with Sjögren’s syndrome)

Diagnosis and other testing for Sjögren’s syndrome
The Schirmer’s test, which measures tear formation, is used to diagnose Sjögren’s syndrome. A slit-lamp test is used to look for corneal damage. Erythrocyte sedimentation rate (ESR) is elevated in 80% of patients. Rheumatoid factor is present in 52% of primary cases and 98% of secondary-type cases. A mild normochromic normocytic anemia is present in 50% of patients, and leukopenia occurs in up to 42% of patients.

Creatinine clearance is diminished in up to 50% of patients. Anti-nuclear antibody (ANA) is positive in 70% of patients. SS-A and SS-B are marker antibodies for Sjögren’s syndrome—70% of patients are positive for SS-A and 40% are positive for SS-B (these also are found in individuals with lupus). Immunoglobulins usually are elevated in Sjögren’s syndrome patients. Rose bengal staining can confirm ocular involvement. A sialogram checks the condition of salivary glands, and salivary scintigraphy measures salivary gland function. A chest x-ray sometimes is ordered to look for lung infection. Lip biopsy often is ordered to detect the presence of inflammatory cell clusters, which can indicate Sjögren’s syndrome. A urine sample is used to see if the kidneys are impacted.

Medical treatment of Sjögren’s syndrome
Medications used for individuals with Sjögren’s syndrome include eye lubricants, salivary substitutes, nasal saline sprays, cholinergic agents (increase activity of exocrine glands), immunosuppressive agents (used for glomerulonephritis, interstitial pneumonitis, and pseudolymphoma), and hydroxychloroquine to treat arthralgias and skin symptoms. Nonsteroidal anti-inflammatory drugs sometimes are used to treat arthritis symptoms. Antifungal drugs are used to treat oral thrush. A minor surgical procedure can seal the tear ducts. Using a dehumidifier, avoiding sitting in front of fans or air-conditioning vents, and wearing goggles or other protective eyewear when outside can help with dry eyes.

Diet and Sjögren’s syndrome
Because Sjögren’s syndrome leads to dental caries, a reduced-sugar diet sometimes is recommended. Frequent brushing and flossing of teeth, regular dental examinations, topical fluoride treatments, and antimicrobial mouthwashes are recommended. Avoid mouthwashes that contain alcohol if the mouth is sore.

Many people with Sjögren’s syndrome have trouble chewing and swallowing because of dry mouth. Some people have pain when eating. The following tips may help:

• Moisten foods with mayonnaise, broths, sauces, gravies, yogurt, salad dressings, etc
• Thin foods with skim milk, broth, water, or melted margarine
• Finely chop or liquefy foods
• Use a straw if it helps with swallowing
• Drink between every bite of food
• Put vegetables on your sandwiches to add moisture
• Choose soft and creamy foods, such as:

Macaroni and cheese
Mashed potatoes
Canned fruit
Pudding
Ice cream

• Consider adding a commercial nutrition supplement, Instant Breakfast® drinks, or homemade milk shakes to the meal plan if you are not eating enough for good nutrition
• Suck on ice pops, ice chips, or sugarless hard candies
• Know that salty, acidic, and spicy foods, as well as carbonated drinks, may make pain worse; these foods include:

Citrus fruits and juices
Hot sauces
Curry dishes
Salsa
Chili peppers
Tomatoes
Ketchup

• Do not eat steaming hot foods—room temperature or cold foods are much easier to tolerate
• Beware of hard and crunchy foods, such as:

Crackers
Chips
Popcorn
Nuts
Bacon

• Understand that certain medications can cause mouth dryness—speak to your pharmacist or physician if this is a concern
• Talk to your doctor about taking vitamin E, which may help with dry mouth
• Avoid alcoholic beverages, which will make the mouth even drier

People experiencing changes in taste and smell may find that the following tips helpful:

• Marinate foods
• Try tart foods and drinks
• Add sugar or sweetener to foods that taste salty, bitter, or acidic
• Experiment with fresh herbs and condiments
• Serve foods at room temperature
• Keep foods and beverages covered—use cups with lids
• Drink through a straw

Gastroesophageal reflux disease (GERD) is common among people with Sjögren’s syndrome. The following tips may help people with GERD:

• Avoid high-fat foods, such as:

Cream
Processed luncheon meats
Fried foods
Nuts
Nut butters
High-fat desserts

• Limit the amounts of oil, margarine, butter, and shortenings used
• Avoid peppermint and spearmint
• Avoid chocolate
• Avoid alcohol and caffeinated beverages
• Know that you may need to avoid other foods, because symptoms vary among individuals
• Wait 3 hours after eating before lying down
• Eat small meals throughout the day

Here are some other interesting fact:

• Some research shows that consuming flaxseed oil may help with dry eye
• 3.3% of patients with celiac disease develop Sjögren’s syndrome (gluten-free diet instruction is needed for patients with celiac disease)
• Fatty fish, fish oil supplements, and vitamin D may help to decrease inflammation
• If methotrexate is used:

Increase folic acid intake either by diet or supplementation
Know that it also can cause anemia, nausea, vomiting, and stomatitis

• Carbohydrate intolerance can result from chronic inflammation and/or steroid usage
• A higher-than-average risk for osteoporosis exists among this population—calcium and vitamin D supplementation sometimes is needed

References and recommended readings

Adams S. Sjogren’s syndrome and celiac disease.
Available at: http://www.celiac.com/articles/105/1/Sjogrens-Syndrome-and-Celiac-Disease/Page1.html.
Accessed January 18, 2011.

Escott-Stump S. Nutrition and Diagnosis-Related Care. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2002:479-481.

Mayo Clinic. Sjogren’s syndrome.
Available at: http://www.bing.com/health/article/mayo-125378/Sjogrens-syndrome?q=sjogren's+syndrome&qpvt=Sjogren+syndrome.
Accessed January 18, 2011.

Phelan D. Sjogren syndrome.
Available at: http://emedicine.medscape.com/article/809290-overview.
Accessed January 18, 2011.

Shiel WC Jr. Sjogren’s syndrome.
Available at: http://www.medicinenet.com/sjogrens_syndrome/article.htm.
Accessed January 18, 2011.

Sjögren’s Syndrome Foundation. Diagnosis.
Available at: http://www.sjogrens.org/home/about-sjogrens-syndrome/diagnosis.
Accessed January 18, 2011.

Sjögren’s Syndrome Foundation. Diet & food tips.
Available at: http://www.sjogrens.org/home/about-sjogrens-syndrome/living-with-sjogrens/diet-a-food-tips.
Accessed January 18, 2011.

Sjögren’s Syndrome Foundation. Symptoms.
Available at: http://www.sjogrens.org/home/about-sjogrens-syndrome/symptoms.
Accessed January 18, 2011.

Review Date 2/11
G-1517