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, causing inflammation in the body and leading to the destruction of tissue. In Sjögren’s syndrome, the infection-fighting cells of the immune system (called lymphocytes) attack the normal cells of exocrine glands – the glands that produce moisture in the eyes, mouth and other tissues. This action damages these glands, making them unable to produce moisture, resulting in dryness and damage.
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 who develop it are 40 years or older. The chances of developing Sjögren’s syndrome are greater if a relative has it or another autoimmune disease.
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 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.
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, such as the airways/throat and the genitals. Other symptoms of Sjögren’s include:
- 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
- Dry cough or hoarseness
- Dryness in nose
- Difficulty speaking
- Difficulty swallowing or eating
- Vaginal dryness
Sjögren’s syndrome can also 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.
Diagnosis
Early diagnosis is important to lessen the effect of Sjögrens on the eyes, mouth and other organs and to reduce damage. The doctor will ask questions about 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 are antibodies that 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 (which signals an immune response) or C-reactive protein (which signals inflammation). An erythrocyte sedimentation rate (ESR or sed rate) test may also be done to check for 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.
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) help reduce inflammation. People with severe Sjögren’s syndrome may receive a corticosteroid , which reduces inflammation in the body, or a disease-modifying antirheumatic drug (DMARD), which suppresses 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:
Prescription medications that stimulate saliva flow include
pilocarpine
tablets or
cevimeline
capsules. 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.
Prescription eye drops, and pellets can help keep eyes moist. Cyclosporine ophthalmic emulsion is an immunosuppressant that ultimately helps increase tear production. Hydroxypropyl cellulose pellets are slow-release tear pellets that may reduce the need for artificial tears. FreshKote artificial tears help redistribute moisture 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, helping them keep their natural moisture.
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.
A doctor may recommend nonsteroidal anti-inflammatory drugs (NSAIDs), such as 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 corticosteroids or stronger DMARDs, such as azathioprine or cyclophosphamide.
A doctor may prescribe antifungal tablets or lozenges to treat this type of infection.
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.
Self Care
Taking a proactive role in treatment, called self-management, is one of the best ways to help manage the symptoms of Sjögren’s syndrome.
- 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 with plain water.
- Avoid sugar-containing foods and drinks between meals.
- 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.
- 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.
- Rest joints when they are swollen and painful to reduce inflammation and fight the fatigue that can come with a flare.
- To relieve joint stiffness and improve range of motion, exercise safely between flares. An exercise program should emphasize low-impact aerobic conditioning, muscle strengthening and flexibility. Talk to a doctor before beginning any exercise program.
Avoid using Vaseline or other oil-based lubricants, which can lead to infections.
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