Sjogren's Syndrome

Sjogren's syndrome diagnosis methods vary but may involve a salivary gland biopsy, blood test, or antigen test. Sjogren's syndrome treatment includes medications, diet, and surgery.

What is the medical definition of Sjögren's syndrome?

• Sjögren's syndrome is a disorder of the moisture-producing glands, such as the tear glands (lacrimal glands) and the salivary glands.
• These glands become infiltrated with white blood cells (lymphocytes) that are part of our immune system. This causes the glands to produce less moisture, leading to dryness of the eyes and mouth.

What does Sjögren's syndrome do to the body?

• In some cases, lymphocytes also infiltrate internal organs such as the lungs, the kidneys, the nervous system, the liver, and the intestines.
• Because these infiltrates can affect multiple organs, they can cause a wide variety of symptoms.
• Sjögren's syndrome often occurs in people who have other rheumatic disorders such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma, or polymyositis/dermatomyositis. This is described as secondary Sjögren's syndrome.
• When the syndrome occurs without another rheumatic disorder, it is called primary Sjögren's syndrome.
• Sjögren's syndrome affects a small portion of the population of the United States. The condition is found throughout the world and in all ethnic groups.
• While Sjögren's syndrome can strike anyone, it most often affects middle-aged and elderly women.

The cause of Sjögren's syndrome is not known. The infiltration of moisture-producing glands by lymphocytes is an autoimmune response. This means that the body's immune system mistakenly attacks the body's own cells. The infiltration of lymphocytes can damage the gland. Precisely what causes this to happen is not known, but it is probably a combination of genetic (inherited) factors and unknown environmental factors.

The defining symptoms of Sjögren's syndrome are dry eyes (xerophthalmia) and dry mouth (xerostomia). Other areas can be dry as well, such as the inside of the nose, the skin, the airways of the lungs, and the vagina. These symptoms often are referred to as the sicca (dryness) complex.

A dry mouth can cause

• difficulty chewing or swallowing;
• inability to eat dry foods, such as crackers, that stick on the roof of the mouth;
• cracked or sore tongue or tongue sticking to the roof of the mouth;
• the dry, burning throat that leads to a dry cough;
• awakening at night with the need for a drink of water;
• difficulty speaking continuously;
• hoarseness;
• high incidence of dental decay and periodontal disease;
• change in the sense of taste;
• difficulty wearing dentures;
• cracks and redness in the corners of the mouth.

Dry eyes can cause

• red, itchy, or painful eyes;
• gritty, scratchy, burning, or sandy sensation in the eyes;
• eyes matted and stuck closed on awakening;
• blurry vision;
• sensitivity to bright light that makes reading or watching television difficult;
• damage to the cornea, the dome over the colored portion (iris) of the eye.

Almost every system of the body can be affected. Symptoms depend on which areas are affected and may include any of the following:

• Painful swelling of the parotid (salivary) glands: These glands are located between your ear and the angle of your jaw.
• Tiredness (fatigue) that can be severe enough to interfere with normal activities
• Joint pain (arthralgia) and occasionally joint swelling (arthritis)
• Dry and itchy skin
• Red-purple skin lesions (palpable purpura) that commonly occur on the legs: These occur when blood vessels in the skin become inflamed
• Episodic white, blue, and red discoloration of the fingers or toes (Raynaud's phenomena)
• Painful sexual intercourse (dyspareunia)
• Dry cough
• Difficulty swallowing
• Recurrent pneumonia and bronchitis
• Acid reflux and heartburn
• Severe abdominal pain that could be due to pancreatitis or kidney stones
• Inflammation of the bladder causes frequent urination, lower abdominal pain, and pain on urination
• Thyroid disease may also accompany Sjögren's syndrome

Symptoms are mild in most people but can be very severe in others. Symptoms can vary over time and may improve, worsen, or even go away completely for periods.

Dry eyes and mouth do not always mean Sjögren's syndrome. The symptoms are common and can be caused by other medical disorders, by some medications, and by anxiety. It is important to consider other diseases that produce similar symptoms. Dryness also may be a result of normal changes in glands and tissues that occur with aging, previous radiation to the head and neck, lymphoma, sarcoidosis, hepatitis C, human immune deficiency virus, human T-cell leukemia virus-1 infection, cancer, inflammatory disease, infections, and medications.

If you have dryness in the mouth, throat, or eyes that persists and is bothersome, you should see your healthcare professional. Swollen or painful parotid glands also warrant a visit to your healthcare professional. Dry, "gritty," or burning eyes warrant a visit to an ophthalmologist.

Because the symptoms of Sjögren's syndrome can be caused by many different disorders, the syndrome is often diagnosed incorrectly or not diagnosed at all.

• To correctly identify the cause of your symptoms, your healthcare provider will ask you many detailed questions about your symptoms, your medical and surgical history, your family history, medications and supplements you take, and your habits and lifestyle.
• A thorough physical examination will try to determine whether your symptoms are due to Sjögren's syndrome or to another disorder and whether internal organs are involved.

Lab tests: There is no one lab test that can confirm the diagnosis of Sjögren's syndrome. Testing will be focused on identifying underlying diseases such as rheumatoid arthritis. These tests will also be used to detect the involvement of various body systems and more serious complications. Your health-care professional may refer you to a rheumatologist who has special expertise in Sjögren's syndrome and related disorders.

• Complete blood cell count (CBC): Blood cell counts are most often normal, but the level of hemoglobin may be low (anemia). A low platelet count or white blood cell count may suggest lupus.
• Blood chemistry will help identify liver, kidney, or electrolyte disturbances.
• Serum protein electrophoresis
• Rheumatoid factor (RF): The test for rheumatoid factor, which is not specific for rheumatoid arthritis, is positive in 80%-90% of people with Sjögren's syndrome. It is also positive in some people with other autoimmune disorders.
• Antinuclear antibodies (ANA): ANA is present in many patients with autoimmune disorders such as systemic lupus erythematosus or Sjögren's syndrome. While many antibodies can cause a positive ANA test, some are common in people with Sjögren's syndrome; these are sometimes called Sjögren's antibodies, anti-Ro/SS-A, and anti-La/SS-B. The results of the ANA tests are positive in about 50%-75% of people with Sjögren's syndrome. The absence of these antibodies does not exclude the disease.
• Thyroid-stimulating hormone: People with Sjögren's syndrome are more likely to have autoimmune hypothyroidism than the general population.
• Hepatitis C antibodies
• Human immunodeficiency virus (HIV) antibodies
• Human T-cell leukemia virus-1 (HTLV-1) antibodies

Salivary gland tests: Several tests can be done to try to determine the cause of mouth dryness.

• Biopsy: This is the single most accurate test for confirming a diagnosis of Sjögren's syndrome. The tissue is usually removed through a tiny incision on the inner lip. The tissue is subjected to tests and looked at under a microscope by a pathologist (a specialist in diagnosing diseases by studying tissues). The pathologist looks for infiltration by lymphocytes.
• Sialography: This is a type of X-ray that uses a contrast medium to highlight details of the parotid glands and the rest of the salivary system. This is especially helpful for finding obstructions or narrowing of the salivary ducts.
• Salivary scintigraphy: This test uses a radioactive tracer to measure saliva production.
• Parotid gland flow (allometry): This test measures the actual amount of saliva produced over a set period of time.
• Ultrasound and magnetic resonance imaging (MRI) to evaluate masses

Eye tests: If you have dry eyes, you will probably be referred to an ophthalmologist (a specialist in eye disorders). This physician may conduct various tests to try to determine the cause of your symptoms and whether there is damage to your eyes.

• Schirmer test: This simple test measures tear production using a strip of filter paper placed on the lower eyelid for five minutes.
• Rose Bengal staining/slit-lamp exam: If you have dry eyes, you will probably be referred to an ophthalmologist (a specialist in eye disorders). This physician may conduct various tests to try to determine the cause of your symptoms and whether there is damage to your eyes.

Other tests: Some symptoms or lab findings may prompt biopsy of other tissues, such as the kidney, the intestine, the lung, or lymph nodes.

There is no known cure for Sjögren's syndrome, nor is there a treatment to restore secretion of moisture by the glands. For the most part, treatment is designed to help relieve symptoms.

If you have Sjögren's syndrome, several different professionals will probably be involved in your care.

• Your primary care provider should always be part of your team.
• Rheumatologists have the most specific training and experience in Sjögren's syndrome as well as the many disorders often associated with the syndrome.
• Ophthalmologists can diagnose early problems with the cornea and assess the degree of damage to the eye. If necessary, they also can perform surgery to help treat or prevent eye damage. They can also help exclude other conditions that cause dry eyes (allergies, contact lens irritation).
• Otolaryngologists (ear, nose, and throat specialists) may be needed if a salivary gland biopsy is necessary to establish a diagnosis. Also, inflammation of the sinuses (sinusitis) occurs more frequently in patients with Sjögren's syndrome.
• Dentists provide appropriate oral care to prevent and treat tooth decay and gingivitis.
• Other subspecialists may be consulted for specific complications of Sjögren's syndrome.

Much of the treatment of Sjögren's syndrome is aimed at relieving symptoms. Your healthcare professional will provide guidance and suggestions, but you can also find treatments that work best for you.

It is important to know whether your symptoms are worse or better at home, at work, or when outdoors as this will assist in identifying which environments need to be modified to improve your symptoms.

Ask your healthcare professional whether any medications you take for other medical problems could be contributing to your dryness. If so, ask him or her about alternatives. Some common medications can worsen dry eyes and dry mouth and need to be avoided.

• Antihistamines
• Decongestants
• Antidepressants
• Diuretics (water pills)
• Tranquilizers
• Some blood pressure medications
• Some diarrhea medications
• Some antipsychotic medications

These general tips may help with dry eyes.

• Blink several times a minute while reading or working on the computer. Lowering the computer monitor below eye level can decrease the width of the eyelid opening and help conserves tears.
• Protect your eyes from windy or breezy conditions.
• Use humidifiers in the rooms where you spend much of your time, including your bedroom. Use distilled water in areas with hard water.
• Don't smoke, and stay away from smoky rooms.
• If you wear eye makeup, apply only to the upper eyelids and to the tips of your eyelashes to keep it out of your eyes.
• Eyeglasses fitted with moisture shields can decrease evaporation.
• Swim or ski goggles are also effective in decreasing evaporation, but wrap-around sunglasses are generally more useful.
• The use of contact lens can aggravate dry-eye symptoms, and their use is also associated with infections.

These general tips may help with dry mouth and its complications.

• Patients with dry mouths can liberally drink sips of water and take bottled water with them on trips. Keep a glass of water at your bedside to moisten your mouth at night.
• A humidifier at the bedside can relieve nighttime dryness. Keep the humidifier clean and change the water daily.
• Chew sugarless gum or suck on sugarless, sour hard candies (especially grape or lemon) to stimulate saliva production.
• Sometimes sucking on a non-nutritive object may increase salivation (such as a cherry pit).
• Avoid mouthwashes that contain alcohol, as they can lead to drying. Instead, rinse your mouth with water several times a day.
• Brush your teeth gently with fluoride toothpaste after every meal and before going to bed. Nonfoaming toothpaste is less drying.
• Avoid sugary foods and snacks that promote tooth decay. When sugary foods are eaten, immediately brush or rinse your teeth.
• If you wear dentures, disinfect them often.
• Look at your mouth every day to check for sores and redness that could signal an infection.

Many of the most widely used preparations for Sjögren's syndrome are available over the counter (without a prescription). Most of these treatments are used on a "trial and error" basis. Often a specific brand of a product will work for an individual while another brand will not work as well. It is worth trying different products to find the ones that work best for you.

Artificial tears

Artificial tears should be applied liberally for dry eyes. These products are safe and may be used as often as needed. They are available without a prescription at pharmacies. They all contain water, salts, thickening agents, stabilizers, and pH buffers. Examples include Celluvisc, Murine, Refresh, and Tears Naturale.

• You may need to apply artificial tears more often if you enter an environment with low humidity (such as air-conditioned rooms and airplanes).
• Artificial-tear preparations with hydroxymethyl cellulose or dextran are more viscous and may last longer before a reapplication is needed. An example is hydroxypropyl methylcellulose (Lacrisert).
• Some artificial-tear preparations contain preservatives to protect against infection. Preservatives may be noxious to the eye surface and can cause a burning sensation when used more often than four times a day. You may want to use a preservative-free preparation to avoid eye irritation if you need to use artificial tears more frequently. These are packaged in one-time-use dispensers. Once the seal is opened, it should not be used again.
• If your eyes are matted closed when you wake up in the morning, try a more viscous preparation, such as Lacri-Lube, at night. While the more viscous preparations can be applied less often, they can make your vision filmy. Therefore, they are best used at night.
• The more viscous preparations can occasionally lead to blepharitis (inflammation of the eyelid), which can make sicca symptoms worse. Important to use only 1/8 inch (3 mm) of the ointment because overuse can block the tear ducts.
• Restasis (cyclosporin A) is a prescription eyedrop treatment. It decreases inflammation in the eye and also allows the gland's function to recover. It may take at least three to six months before you notice any improvement.
• With advanced disease, other therapies include antibiotics, autologous (derived from patient's own body) serum, systemic anti-inflammatory therapy including acetylcysteine, and topical vitamin A.

Artificial saliva

Artificial saliva can be used as needed for dry mouth. Some people do not tolerate these preparations very well. Try different products, such as Salivart, Saliment, Saliva Substitute, MouthKote, and Xero-Lube, to find one that works for you.

• See your dentist regularly. You may need to have preventive treatments more often than the standard six months. Your dentist may advise fluoride treatments. Use toothpaste without detergents to reduce mouth irritation. Brands include Biotene toothpaste, Biotene mouth rinse, Dental Care toothpaste, and Oral Balance gel.
• A dry mouth increases your susceptibility to oral infections. Watch for thrush (redness with overlying white patches) and tender sores that don't heal quickly. If you develop these conditions frequently, keep topical antifungal agents, such as nystatin troches, on hand and use them as needed. Fluconazole (Diflucan), an antifungal medication taken in pill form, may be needed occasionally.
• Seek treatment for sinusitis or blocked sinuses, because these problems may contribute to mouth breathing, which can worsen drying of the mouth.
• A humidifier may help keep your mouth moist.
• Systemic medications that can increase salivation may be needed if topical therapy does not work.

Other treatments

Skin, nose, and vaginal dryness often can be relieved with topical treatments.

• Skin creams, such as Eucerin, or lotions, such as Lubriderm, can help with dry skin.
• Saline nasal sprays can help keep the inside of the nose moist. Decongestant sprays should be avoided, as these can worsen the dryness.
• Vaginal lubricants, such as Replens, may be used to treat vaginal dryness.
• Postmenopausal women can use vaginal estrogen creams in some cases.
• Women with Sjögren's syndrome may be susceptible to vaginal yeast infections, which should be treated promptly.
• Reflux (heartburn) is common in people with Sjogren's and is treated similarly to other people.
• Fatigue may be a symptom on its own or accompanied by vague symptoms of poor concentration, muscle aching, and impaired memory. This may suggest fibromyalgia. See the treatment section of fibromyalgia.

Systemic treatment

Drugs that increase saliva secretion: These can be used if topical therapy is not sufficient to treat dry mouth. Examples include pilocarpine (Salagen) and cevimeline (Evoxac). The most common adverse effect is increased sweating (in as many as 29%). Some studies suggest that interferon-alpha may be a useful therapy in the future.

Nonsteroidal anti-inflammatory drugs (NSAIDs): These agents reduce inflammation and mild to moderately severe pain. Examples are ibuprofen (Advil), naproxen (Naprosyn), and celecoxib (Celebrex). These agents should be used with caution if you have aspirin sensitivity or kidney disease. If you are older than 65 years of age, have ever had peptic ulcer disease, use steroids or blood thinners, smoke cigarettes, or drink alcohol, talk to your healthcare provider about alternatives. Long-term use of these medications has been linked to an increased risk for myocardial infarctions.

Inflammation of the upper airways (larynx, trachea, and bronchi) can be treated with mucus-thinning agents like guaifenesin.

Disease-modifying antirheumatic drugs: These drugs treat rheumatoid arthritis and other rheumatic conditions that do not get better with NSAIDs. Examples include hydroxychloroquine (Plaquenil), azathioprine (Imuran), and methotrexate (Rheumatrex). Newer biological agents are being studied for the treatment of Sjögren's syndrome.

Immunosuppressive drugs: These agents may be suitable for people with Sjögren's syndrome who develop a major organ manifestation such as interstitial lung disease. Examples include prednisone (cortisone), methotrexate, cyclophosphamide (Cytoxan), azathioprine (Imuran), and mycophenolate mofetil (CellCept). These medications have a number of different side effects that should be discussed carefully with your healthcare provider. If you take one of these medications, you may have to have regular blood tests to monitor you for possible side effects.

Recent research studies suggest that rituximab (Rituxan) may be beneficial in certain forms of Sjögren's syndrome. Rituximab works by decreasing the number of B cells, which are specialized white blood cells that are involved in the pathophysiology of Sjögren's syndrome. Belimumab (Benlysta) is also showing promise in the treatment of Sjögren's syndrome. It is also an intravenous medication used in systemic lupus erythematosus that affects the activity of overactive immune-mediating cells.

Surgery does not have a major role in the treatment of Sjögren's syndrome. Blockage of lacrimal puncta (punctual occlusion) to help retain tears in the eyes is one approach that helps some people.

If you need anesthesia for any reason, inform the anesthesiologist of your diagnosis. Under general anesthesia, there is an increased risk of mucous plugs in the airways after surgery. Medications used during surgery can also further dry the airways. Your anesthesiologists can take special measures to avoid these complications.

The professional coordinating your care will want to monitor your progress at regular follow-up visits. This visits should be scheduled about every three months or, if your condition is stable, every six months. If you are having active problems or concerns about an emerging related illness, you may be seen more often.

There is no known way of preventing Sjögren's syndrome.

Most people with Sjögren's syndrome have no major organ involvement and do well. For people who have related conditions as well as Sjögren's syndrome, the outlook is tied more closely to the related condition than to Sjögren's syndrome. Sjögren's syndrome does, however, have potentially serious complications. They include the following:

• Lymphoma: This is cancer of the lymphatic system, which includes lymph nodes. Some research has shown that the risk of developing lymphoma is higher in people with Sjögren's syndrome than in the general population. The average time between diagnosis of Sjögren's syndrome and appearance of the lymphoma is seven and a half years. The most common symptom of lymphoma is a large mass or swelling in the neck, under an arm, or in the groin. Other symptoms include pain, numbness, or tingling in an arm or leg, fatigue, unexplained fevers or night sweats, and unexplained weight loss.
• Infection of the parotid gland: This usually is signaled by swelling, pain, redness, and warmth on one side of the face, typically along the jawline. Heat and massage, locally applied to the parotid glands on the sides of the jaw, can help and prevent this complication.
• Parotid tumors: One of the parotid glands becomes enlarged and unusually hard.
• Problems in offspring: Children born to women with Sjögren's syndrome have greater-than-normal risks for newborn lupus and congenital heart block.

Living with the effects of Sjögren's syndrome can be difficult. Sometimes you will feel frustrated, perhaps even angry or resentful. Sometimes it helps to have someone to talk to.

This is the purpose of support groups. Support groups consist of people in the same situation you are in. They come together to help each other and to help themselves. Support groups provide reassurance, motivation, and inspiration. They help you see that your situation is not unique, and that gives you power. They also provide practical tips on coping with this disorder.

Support groups meet in person, on the telephone, or on the Internet. To find a support group that works for you, contact the following organization or look on the Internet. If you do not have access to the Internet, go to the public library.

• Sjögren's Syndrome Foundation
  800-475-6473 (voice mail only)

National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health, Questions and Answers about Sjögren's Syndrome

National Institute of Dental and Craniofacial Research (NIDCR),

National Institutes of Health, Sjögren's Syndrome

National Institute of Neurological Disorders and Stroke (NINDS),

National Institutes of Health, , NINDS Sjögren's Syndrome Information Page

Sjögren's Syndrome Foundation