Sjogren’s syndrome (SS) is a chronic autoimmune disorder in which the moisture-producing glands do not function correctly; SS can also affect internal organs.

Sjogren’s syndrome is a relatively common disease, although often under-diagnosed. Sjogren’s syndrome can occur alone or in association with other autoimmune diseases, most commonly lupus and rheumatoid arthritis (RA).

Several studies have shown various differences between people with SS alone and those with SS and another connective tissue disease. Because of these differences, Sjogren’s syndrome is termed primary Sjogren’s syndrome when it occurs by itself and secondary Sjogren’s syndrome when associated with another connective tissue disease.

The diagnostic criteria for Sjogren’s syndrome include:

• dry mouth
• poor salivary (saliva-producing) gland production
• dry eyes
• often the presence of antinuclear antibodies
• the presence of a positive rheumatoid factor.

Because the disease is mild in many people, the first signs of mucosal dryness may be present for years before the disease becomes clearly evident.

These symptoms and signs may be associated with lung involvement, kidney involvement, and vasculitis (inflammation of blood vessels). In addition, lymphomas (tumors of the lymph glands), will develop in 6 percent of people with primary SS who have systemic disease.

Laboratory abnormalities in Sjogren’s Syndrome

Autoantibodies are common in SS.

• 80 percent of those with SS test positive for antinuclear antibodies (ANA).
• Rheumatoid factor is present in 75- 95 percent of those with SS.
• Elevated protein levels will be seen in 80 percent of those with SS.
• Other nonspecific laboratory abnormalities that are commonly noted in SS are elevated erythrocyte sedimentation rates (a sign of inflammation), mild anemia, and low albumin levels.

Certain genes may also be found more frequently in people with primary SS. These "histocompatibility antigens" may include HLA-B8 and HLA-DR3.

Diagnostic tests and procedures in Sjogren’s Syndrome

Several tests are commonly used to confirm a suspected diagnosis of SS:

• In the Schirmer's test, a piece of filter paper is placed in the corner of the eye to measure the degree of wetting after five minutes.
• The Rose-Bengal staining test determines whether there is inflammation of the cornea.
• Salivary gland flow rates help to determine whether there is decreased saliva production.
• Salivary gland biopsy of the lip or parotid gland may help to establish the diagnosis.

Associations of Sjogren’s Syndrome with lupus and other disorders

The coexistence of Sjogren’s syndrome with lupus was first described in 1959. Since that report, numerous studies have compared and contrasted the two diseases. Many of the clinical and serological (blood-related) features of SS and lupus make the precise diagnosis difficult because there are similarities between the two diseases. It is not uncommon to find features of secondary SS in people with lupus. People with primary SS and people with lupus have certain similar disease symptoms, including arthritis, skin rash, and kidney disease. Increased levels of antinuclear antibodies are also seen in both diseases.

In some cases, an increased association of certain genes is found in individuals with both primary SS and lupus.

Sjogren’s syndrome may also be confused with disease processes, such as viral infections and drug side effects (dryness from various antihypertensive agents, antidepressant agents or antihistamine agents, etc.).

Other disease processes may also cause salivary gland enlargement are viral infections, sarcoidosis, and diabetes.

Treatment for Sjogren’s Syndrome

Treatment for Sjogren’s syndrome attempts to relieve the effects of chronic dryness of the mouth and eyes by increasing the lubrication and moisturization of the affected tissues:

• Fluid intake is often necessary both with and between meals because of the decrease in salivary gland secretion.
• Artificial tears in various forms help replace decreased tear production. However, artificial tears containing preservatives may cause irritation.
• Plugging the lacrimal (tear) ducts with temporary or longer lasting collagen plugs, can be very effective in increasing moisture of the conjunctiva and cornea. Various other experimental agents are being developed.
• Local oral agents have been used for continuing lubrication, but with limited success.
• Systemic agents have been used, with varied success, including pilocarpine (Salagen®), 5 mg three times a day, or cevimiline (Evoxac®), 30 mg three times a day.
• Hydroxychloroquine (Plaquenil®), an antimalarial, has been used for the fatigue and joint pain associated with primary SS, and also to decrease levels of some of the autoantibodies and sedimentation rates.
• Systemic corticosteroids and/or immunosuppressive agents (cytotoxic drugs) have been used for various extraglandular symptoms of SS, such as: vasculitis, lung involvement kidney involvement. However, cytotoxic agents should be used with great care as they may increase the risk of lymphoma.
• Biologic agents have not been shown to improve the signs and symptoms of SS in studies thus far.

Prognosis of Sjogren’s Syndrome

The overall disease course of Sjogren’s syndrome can vary from very mild to fairly significant. Individuals with secondary SS seem to have milder disease, as compared to those with primary SS. As previously noted, people with primary SS seem to have a greater chance of extraglandular involvement.

Early diagnosis and early treatment are extremely important in trying to prevent damage to major organs.

Learning as much as possible about the disease process will enable individuals with commonly associated diseases to be aware of problems and symptoms.

Routine follow-up with the physician is equally important.

The Lupus Foundation of America would like to thank Stuart Kassan, MD, for providing this information.