What is Sjögren’s Syndrome?
Sjögren’s syndrome is a chronic, autoimmune disease that causes dryness of the eyes, mouth and other body parts.
In an autoimmune disease, the immune system mistakenly attacks healthy tissue, leading to inflammation in the body. In Sjögren’s syndrome, the infection-fighting cells of the immune system (lymphocytes) attack the normal cells of glands that produce moisture in the eyes, mouth and other tissues. These are called exocrine glands. This action damages these glands, making them unable to produce moisture.
The disease is classified either as:
- Primary Sjögren’s. The condition exists by itself.
- Secondary Sjögren’s. It develops as complication of a pre-existing disease such as rheumatoid arthritis, lupus, scleroderma or myositis
Sjögren’s syndrome affects one to four million people in the United States. Ninety percent of people with the condition are women. Most people are 40 years or older when diagnosed. The chances of developing Sjögren’s syndrome are greater if a relative has it or another autoimmune disease.
Sjogren’s Syndrome Symptoms
Dry eyes and mouth (also known as sicca syndrome) are the most common signs of Sjögren’s, but the disease may affect other parts of the body. Other symptoms of Sjögren’sinclude:
- Itching, burning and redness
- Feeling something is in the eye
- Sensitivity to light
- Thick or stringy saliva
- Swollen salivary glands (located under the tongue, in the cheeks and the jaw)
- Mouth sores or pain
- Loss of sense of taste
- Yeast infection in the mouth
Nose, Throat and Airways
- Dry cough or hoarseness
- Dryness in nose
- Difficulty speaking
- Difficulty swallowing or eating
- Vaginal dryness
Sjögren’s syndrome also can cause swollen or painful joints, muscle pain or weakness, dry skin, rashes, brain fog (poor concentration or memory), numbness and tingling sensations in the arms and legs due to nerve involvement, heartburn, kidney problems and swollen lymph nodes.
Sjögren’s Syndrome Causes
The cause of Sjögren’s syndrome is unknown. Researchers think that a combination of environmental and genetic factors determines who develops the disease. While there are certain genes that increase a person’s risk for Sjögren’s syndrome, the genes do not act alone. It is believed that in order for a person to develop Sjögren’s, the immune system must be activated by some sort of trigger – such as a viral or bacterial infection – that sends the immune system into overdrive.
Evidence also suggests that if a relative has Sjögren’s syndrome, a person has a higher risk for it. In fact, about 12 percent of people with it have one or more relatives with the disease.
It's also common for relatives of people with Sjögren’s syndrome to develop other types of autoimmune disease such as lupus or hypothyroidism.
While there isn’t yet a concrete answer as to why Sjögren’s syndrome affects women more often than men, researchers believe the hormone estrogen might play a role. A major risk factor for developing Sjögren’s syndrome is being a post-menopausal woman. Estrogen levels in the body drop after menopause.
Sjögren’s Syndrome Diagnosis
Early diagnosis is important to lessen the effect of Sjögrens on the eyes, mouth and other organs. The doctor will ask questions about the person's medical history and discuss symptoms. During the physical examination, the doctor will check for:
- Changes in the eyes, mouth and salivary glands
- Joint inflammation
- Muscle weakness
- Swelling of lymph nodes in the neck
The doctor may recommend an eye examination to be done by an ophthalmologist (eye specialist).
Several tests help the doctor make a diagnosis. They include:
- Blood tests. Some people have specific proteins in their blood called autoantibodies, which react to the body’s own tissues. Certain blood tests can detect these. The anti-SSA (also called anti-Ro) or anti-SSB (also called anti-La) blood tests are the most specific ones for Sjögren’s syndrome. Other autoantibody tests include antinuclear antibody (ANA) and rheumatoid factor (RF). Blood tests may also be done to look for signs of inflammation, such as increased levels of immunoglobulin or C-reactive proteins (CRP). These are both proteins that signal immune reactions. An erythrocyte sedimentation rate (ESR or sed rate) test may also be done to check for general signs of increased inflammation.
- Dry eye tests. The Schirmer test is used to determine how well the eyes produce tears. A slit-lamp test checks the surface of the eye (cornea) for dryness-related damage.
- Saliva tests. The doctor will measure saliva output, how well salivary glands function and whether there is inflammation.
- Chest X-ray. Because Sjögren’s can affect the lungs, the doctor may order this test to check for inflammation.
Sjögren’s Syndrome Treatment
There is no cure for Sjögren’s syndrome. As with other autoimmune diseases, the severity of Sjögren’s varies from person to person. Many patients have a mild disease that only affects the eyes and mouth. Others have symptoms that wax and wane in severity, or may even go into remission. Some have severe and chronic (long-term) symptoms.
Current treatments focus on managing the symptoms. Moisture replacement therapies help relieve dryness and nonsteroidal anti-inflammatory drugs (NSAIDs) to control inflammation. People with severe Sjögren’s syndrome may receive corticosteroids, which mimic hormones that fight inflammation in the body, or disease-modifying antirheumatic drugs (DMARDs), which suppress the body’s immune response. In addition to a primary care physician, other members of the healthcare team may include a rheumatologist, an ophthalmologist and a dentist.
Here are some of the treatments and therapies for different areas of the body affected by Sjögren’s syndrome:
For Dry Mouth
Prescription medications that stimulate saliva flow include pilocarpine tablets or cevimeline capsules help treat symptoms of dry mouth by stimulating the salivary glands. The effects only last a few hours, so you may need to take several doses each day. People with asthma or narrow angle glaucoma should ask a doctor if it’s safe to take these medications.
Saliva substitutes or mouth-coating gels can be helpful, especially at night when the mouth becomes drier. These are available in sprays, liquids and pretreated swabs. Over-the-counter options include Glandosane, MedActive, MoiStir, MouthKote, Oasis, Optimoist, Oralube, Salivart and Xero-Lube. A doctor may also prescribe Aquoral mouth spray or NeutraSal mouth rinse.
For Dry Eyes
Some prescription eye drops or pellets can help keep eyes moist. Cyclosporine ophthalmic emulsion and hydroxypropyl cellulose pellets are slow-release tear pellets that may reduce the need for artificial tears. FreshKote artificial tears help redistribute moisture in damaged cells on the eyes’ surface. Some nonprescription artificial tears are Refresh, TheraTears and GenTeal. These products contain different ingredients and can be used individually or together. In more severe cases of dry eyes, the doctor may recommend a surgery called punctal occlusion. This operation keeps tears from draining out of your eyes and into the nose, helping the eyes keep their natural moisture.
For Dry Mouth, Throat and Upper Airways
Prescription medicines for these symptoms include cevimeline, pilocarpine, Linseed extract, Sorbitol or malic acid. They are available in a pill, liquid, and lozenge form. Using an expectorant such as guaifenesin may help with dryness of the throat and respiratory tract.
For Joint Pain
A doctor may recommend nonsteroidal anti-inflammatory drugs (NSAIDs), including aspirin, ibuprofen and naproxen, to decrease joint pain and stiffness and ease muscle aches. Disease-modifying antirheumatic drugs (DMARDs), such as hydroxychloroquine or methotrexate, may be prescribed for people to help relieve rashes, fatigue and joint pain. If the muscles, nerves, kidneys or lungs are affected, a doctor may recommend stronger DMARDs, corticosteroids, or medicines called azathioprine or cyclophosphamide.
For Oral Yeast Infection
A doctor may prescribe antifungal tablets or lozenges to treat this type of infection.
For Vaginal Dryness
Water-based vaginal lubricants (K-Y Jelly, Astroglide, Replens, Luvena) can ease vaginal dryness and painful intercourse. Estrogen creams or other preparations may be helpful for women who have vaginal dryness due to reduced estrogen levels related to menopause.
Sjögren’s Syndrome Self Care
Taking a proactive role in treatment, called self management, is one of the best ways to help manage the symptoms Sjögren’s syndrome. Here are some tips to do that:
For Dry Mouth and Dental Health
- Take small sips of water throughout the day.
- Chew sugar-free gum or suck on sugar-free hard candies or lozenges, such as Salese or Dentiva, to stimulate saliva flow.
- Use products that contain the artificial sweetener xylitol to help prevent tooth decay.
- Have frequent dental checkups.
- Use an electric toothbrush.
- Ask a dentist to recommend fluoride products specifically for dry mouth.
- Brush and floss teeth regularly, especially after meals. If daytime brushing isn't possible, rinse the mouth with plain water.
- Avoid sugar-containing foods and drinks between meals.
For Dry Eyes
- Use eye drops on a regular basis; try single-unit preservative-free drops.
- Limit the use of drops with preservatives, which can cause dryness and irritation.
- Use lubricating eye ointments or gels at night.
- Use wraparound sunglasses or moisture shields that attach to glasses to prevent loss of moisture from the eyes.
For Dry Nose, Throat and Upper Airways
- Use nasal sprays of water or saline during the day. In some climates, using a home humidifier may help.
- Use water-based nasal gels at nighttime.
For Joint Pain
- Rest joints when they are swollen and painful to help reduce inflammation and fight the fatigue that can come with a flare.
- Exercise safely to relieve joint stiffness and improve range of motion between flares. Talk to a doctor before beginning an exercise program, which should emphasize low-impact aerobic conditioning, muscle strengthening and flexibility.
For Vaginal Dryness
- Avoid using Vaseline or other oil-based lubricants, which can lead to infections.