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What Is Sjögren's Syndrome?

What Is Sjögren’s Syndrome?
Risk Factors 
Why Your Sex Matters
Symptoms
Diagnosis
Treatment
Future approaches to Sjögren’s Syndrome

What Is Sjögren’s Syndrome?
As many as four million Americans about 90 percent of them women may have Sjögren’s syndrome, in which the immune system attacks the body’s moisture-producing glands and tissues. These include the lacrimal glands that produce tears and the salivary glands, as well as mucus membranes in the nose and vagina. In some cases, Sjögren’s can also affect the kidneys, gastrointestinal (GI) tract, lungs, liver, and blood vessels. In 50 percent of cases, Sjögren’s syndrome occurs alone (primary Sjögren’s), and 50 percent of the time it occurs in the presence of another autoimmune disease such as RA, lupus, scleroderma, or thyroid disease.

Sjögren’s develops when moisture-producing glands are damaged by activated immune cells, making them less able to respond to signals from the brain to produce tears or saliva. In addition, inflammatory cytokines may damage the nerves and neurotransmitters (chemical substance in the brain) that stimulate the tear ducts or salivary glands. The dry eyes that characterize Sjögren’s are also caused by damage to tiny glands behind the eyelashes (called meibomian glands) that secrete an oil that prevents tears from evaporating too quickly.

Risk Factors 
Like RA and other autoimmune disorders, Sjögren’s can also run in families. Although it can develop at any age, it is most prevalent after age 40. Like other autoimmune diseases, Sjögren’s syndrome appears to be influenced by genetic vulnerability, environmental triggers (such as the Epstein-Barr virus), and hormones.

Why Your Sex Matters
The underlying cause of evaporative dry eye may be low levels of male hormones. Androgens decline after menopause, although not to the extent that estrogen production drops. Researchers are studying androgen therapy as a treatment for primary Sjögren’s and androgen eye drops for dry eye symptoms. Studies have shown that estrogen therapy appears to worsen dry eye.

Women with Sjögren’s syndrome often experience vaginal dryness, which can lead to painful intercourse. Vaginal lubrication does not come from moisture-producing glands but from fluid that is passed from the bloodstream through the vaginal walls. Vaginal dryness in Sjögren’s patients is often mistaken for menopause-related vaginal atrophy or degeneration. Symptoms related to dryness can be helped by moisturizers. Lubricants can make sex more comfortable. Local estrogen therapy in the form of creams, a ring, or suppository tablet  also help dryness by reversing the atrophy of vaginal tissues that occurs after menopause.

Symptoms 
The hallmarks of  Sjögren’s syndrome are dry eyes, dry mouth, and fatigue. Classic dry eye symptoms include a dry, gritty, or burning sensation; itching; light sensitivity; eyelids that stick together; and mucus accumulation in the corners of the eyes on awakening. Women with dry mouth may have difficulty chewing or swallowing, burning mouth or tongue, and increased dental decay (due to low saliva production). Sjögren’s can also cause swollen parotid glands (the largest of the salivary glands, in front of the ears), joint pain, dry nose, and fatigue and can lead to peripheral nerve problems, lung inflammation, and kidney dysfunction. Women with Sjögren’s may also have a slightly increased risk of lymphoma, a malignancy of the lymph glands, so swollen lymph nodes should be investigated.

Diagnosis 
Because symptoms of Sjögren’s syndrome, such as joint pain and fatigue, may overlap with those of RA and other autoimmune diseases, it is often misdiagnosed or missed altogether. Physicians may treat dry eye and overlook other symptoms, such as dry mouth or dry vagina, which may signal Sjögren’s. Dentists may see women with dry mouth and numerous cavities and never ask if they suffer dry eyes as well. A woman may visit her doctor for menopausal symptoms in her late 40s, and not be diagnosed with Sjögren’s syndrome until much later. In fact, the average time from the onset of symptoms to a diagnosis can sometimes exceed six years.

During a work-up, blood tests are performed to check for specific autoantibodies, including RF and antinuclear antibodies (ANAs), present in 70 percent of patient with Sjögren’s, as well as variety of autoimmune diseases including lupus. Your sedimentation (SED) rate will be measured, and you will be given tests to look for elevations in normal blood proteins known as immunoglobulins, common in people with Sjögren’s syndrome.

For women with dry eyes, special tests are performed to measure tear production and the volume of tears produced. The most common of these is the Schirmer’s test, in which small pieces of filter paper are placed between your eyeball and lower lid to measure the amount of moisture produced within five minutes. A slit-lamp examination uses a special lamp and a magnifying device to reveal the normal layer of tears along the lower eyelid, which may appear thickened. Another test, called rose bengal staining (involving a harmless vegetable dye), helps to determine the quality of the oily layer of tear film and its distribution over the surface of the eye. Other tests may be done to assess the condition of the cornea.

A dentist or oral pathologist can measure saliva production in dry mouth. This is done by putting an acidic or sour substance in the mouth to stimulate saliva production. In some cases, a biopsy of the tiny salivary glands in the lower lip may be needed to detect immune cell infiltration.

Treatment 
The symptoms of Sjögren’s syndrome are treated individually. Over-the-counter (OTC) and prescription medications are available to relieve the dry eye and dry mouth symptoms. OTC products include preservative-free artificial tears, artificial saliva, saline nasal sprays, and vaginal lubricants. An ophthalmologist may recommend eye drops containing cyclosporine for dry eye. In some cases, tiny silicone plugs (punctal plugs) are inserted in the tear ducts to prevent tears from draining away. Non-drug strategies include the use of a humidifier and protective goggles to prevent tear evaporation. 

Two oral prescription drugs are used to treat dry mouth, pilocarpine hydrochloride and cevimeline, which are taken after meals to improve salivary flow. Studies suggest they may also improve dry eye and dryness in other areas of the body. Artificial saliva can provide topical relief for dry mouth.

Other manifestations of Sjögren’s, such as kidney or lung problems, are treated separately with medication.

Future Approaches to Sjögren’s Syndrome
One promising avenue of therapy for dry eye is eye drops containing androgens. The National Institutes of Health is also exploring the use of androgens, in the form of dehydroepiandrosterone (DHEA), to treat primary Sjögren’s syndrome. Some medications used to treat RA may also be useful in Sjögren’s. 

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