What do I need to know about Sjögren’s Syndrome and lupus?
Sjögren’s syndrome (SS) is a chronic autoimmune disorder in which the moisture-producing glands that do not function correctly; SS can also affect internal organs.
Sjögren's syndrome is a relatively common disease, although often under-diagnosed. Sjögren'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, Sjögren's syndrome is termed primary Sjögren's syndrome when it occurs by itself and secondary Sjögren's syndrome when associated with another connective tissue disease.
The diagnostic criteria for SS 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.
Symptoms of Sjögren's Syndrome
The symptom most associated with SS 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. Enlargement of parotid or other major salivary glands occurs in two-thirds of those with primary SS, but is uncommon in those with secondary SS.
Eye involvement is the other major manifestation of SS. 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
- 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 primary SS, but rarely in those with secondary syndrome.
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 primary SS who have systemic disease.
Laboratory Abnormalities in Sjögren'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 Sjögren'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 Sjögren's Syndrome with Lupus and Other Disorders
The coexistence of Sjögren'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.
Sjögren'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 Sjögren's Syndrome
Treatment for Sjögren'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 Sjögren's Syndrome
The overall disease course of Sjögren'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, M.D., for this information.
Medically reviewed on July 11, 2013