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What You Need to Know About Sjogren’s Disease
Sjogren’s disease is a chronic autoimmune disorder in which the moisture-producing glands do not function correctly; Sjogren's can also affect internal organs.
Sjogren’s disease is a relatively common disease, although often under-diagnosed. Sjogren’s can occur alone or in association with other autoimmune diseases, most commonly lupus and rheumatoid arthritis (RA).
The diagnostic criteria for Sjogren’s disease 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.
The symptom most associated with Sjogren's is oral (mouth) dryness, known as xerostomia. Other oral symptoms may include:
- burning of the tongue
- cracking of the tongue
- increased dental caries (cavities)
- trouble swallowing
- difficulty speaking without the use of continued lubrication.
- The parotid glands (major salivary glands located behind the jaw) may be enlarged and painful swelling may also be noted.
Eye involvement is the other major manifestation of Sjogren's. People often complain of a sandy or gritty feeling in their eyes, especially in the morning. Other ocular symptoms that may be present include:
- decreased tearing
- redness
- itching
- photosensitivity, due to the loss of the lining cells of the conjunctiva, a condition called keratoconjunctivitis sicca
- A decrease in mucous gland secretion of the upper and lower respiratory tract will be indicated by a chronic dry cough.
- A decrease in mucous gland secretions of the gastrointestinal tract may be associated with esophageal (throat) dryness, as well as trouble swallowing and irritation of the stomach.
- Vaginal dryness may cause irritation and pain during intercourse.
Symptoms that occur outside the glands are seen in one-third of people with Sjogren's disease.
These extraglandular symptoms may include:
- joint pain
- muscle pain
- low-grade fevers
- increased fatigue.
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 Sjogren's who have systemic disease.
Laboratory abnormalities in Sjogren’s disease
Autoantibodies are common in Sjogren's disease.
- 80 percent of those with Sjogren's test positive for antinuclear antibodies (ANA).
- Rheumatoid factor is present in 75- 95 percent of those with Sjogren's.
- Elevated protein levels will be seen in 80 percent of those with Sjogren's.
- Other nonspecific laboratory abnormalities that are commonly noted in Sjogren's 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 Sjogren's. These "histocompatibility antigens" may include HLA-B8 and HLA-DR3.
Diagnostic tests and procedures in Sjogren’s disease
Several tests are commonly used to confirm a suspected diagnosis of Sjogren's:
- 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 disease with lupus and other disorders
The coexistence of Sjogren’s disease 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 Sjogren's and lupus make the precise diagnosis difficult because there are similarities between the two diseases. It is not uncommon to find features of Sjogren's in people with lupus. People with Sjogren's 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 Sjogren's disease and lupus.
Sjogren’s disease 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 disease
Treatment for Sjogren’s disease 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 Sjogren's, 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 Sjogren's disease, 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 Sjogren's in studies thus far.
Prognosis of Sjogren’s disease
The overall disease course of Sjogren’s disease can vary from very mild to fairly significant.
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.