What is it?

Overview

What Is It?
Sjogren’s syndrome is an autoimmune disorder in which the glands that produce moisture are destroyed. The hallmark symptoms are dry eyes and dry mouth.Sjogren's syndrome is a chronic, incurable disorder in which immune system cells attack and destroy the glands that produce moisture. Women are nine times more likely than men to develop Sjogren's (pronounced "show-grins"), which can occur at any age, but a woman is at higher risk for developing the condition after menopause. The Sjögren's Syndrome Foundation estimates that four million Americans suffer from this disorder; many go undiagnosed.

Named after Swedish ophthalmologist Henrik Sjogren, Sjogren Syndrome is an autoimmune disorder, a condition in which the immune system attacks the body's healthy tissues. Ordinarily, the immune system produces antibodies and cells that target such destructive material as viruses and bacteria. In the case of Sjogren's and other autoimmune diseases, the immune system produces autoreactive cells and autoantibodies, which turn against the part of the body they should protect.

Types of Sjogren Syndrome: Primary and Secondary
Sjogren's syndrome may occur alone (referred to as primary Sjogren's syndrome), or it may be associated with other autoimmune/rheumatic diseases, such as rheumatoid arthritis, lupus, polymyositis and scleroderma. When Sjogren's occurs with another autoimmune disease it is called secondary Sjogren's syndrome.

Symptoms of Sjogren Syndrome

Common Symptoms
The hallmark symptoms of Sjogren's are dry eyes and dry mouth. The disorder may also cause skin, nose and vaginal dryness, and it can affect other organs of the body as well, including your skin, lungs, gastrointestinal tract, liver, pancreas, kidneys, thyroid, joints, muscles, blood, blood vessels, brain, spinal cord and peripheral nerves. Inadequate tear production can result in eye irritation and a feeling of having grit or sand in your eyes.

Symptoms vary, as this is a disease of many manifestations. The symptoms may level out, worsen or go into remission (disappear for a while). Some people experience only the mild symptoms of dry eyes and mouth, while others experience cycles of good health followed by severe disease. Many patients are able to treat problems symptomatically, but others must cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands (the salivary glands near the ears), hoarseness, difficulty in swallowing and eating, bronchitis and other pulmonary disorders, debilitating fatigue and joint pain.

Severe Symptoms
In severe cases, Sjogren's can damage vital organs. Also, in one study, the incidence of lymphoma, cancer of the lymphocyte, was 44 percent higher in people with Sjogren's.

Impact of Sjogren Syndrome on Salivary Glands
Salivary glands are a prime target of Sjogren's. They are composed of two parts, the acinar region or secretory end piece and the ductal region. Acinar cells are the site of fluid secretion, and they are responsible for making and secreting more than 85 percent of the proteins involved in producing saliva. Ductal cells cannot secrete fluid. In Sjogren's, autoreactive lymphocytes (a type of white blood cell) infiltrate and destroy the glandular tissue.

Dry Mouth
The lack of saliva generally causes dry mouth (xerostomia); this can lead to difficulties with speech and swallowing food, extensive tooth decay, tooth loss, oral sores and fungal infections in the mouth. If dry mouth is troublesome at night, it can cause sleep problems through a cycle of increased fluid intake and increased urination. The salivary glands may enlarge to produce visible swelling, particularly below the ears at the angle of the jaw; this swelling may be confused with mumps.

Oral Bacteria
Another problem is that the quality of the saliva that is produced is not as good, predisposing you to problems related to oral bacteria. Saliva is the main protective factor for the upper gastrointestinal tract—the mouth, pharynx and esophagus. Salivary proteins help prevent tooth decay; repair wounds or ulcers in the soft mucous membrane tissues; kill bacteria, certain fungi and some viruses; and permit proper swallowing of food. Saliva also facilitates your ability to taste and to speak. All of these functions can be affected by Sjogren's.

Risks for Pregnant Women with Sjogren Syndrome
An autoantibody to the protein SS-A/Ro, found in women with Sjogren's syndrome, can very rarely be associated with congenital heart block and neonatal lupus in newborn babies. If you have Sjogren's syndrome and plan to become pregnant, see your health care professional about testing for this marker and deciding what to do if the marker is present.

Diagnosis

Diagnosis

No single gene, microorganism or hormone is known to cause Sjogren's syndrome. As a result, diagnosis is often difficult. Its symptoms can mimic those of other diseases, and no two people with Sjogren's syndrome have exactly the same set of symptoms or medical history. Moreover,individual symptoms may be reported to different health care professionals—such as dry mouth to the dentist or dry eyes to an optometrist—so the condition often goes undiagnosed. In fact, the average time from the onset of Sjogren's symptoms to diagnosis is over six years.

Once Sjogren's is suspected, a detailed history and physical exam, blood tests for autoantibodies, tests to determine the degree of dry eye and mouth, and sometimes lip biopsies, can point to the presence of Sjogren's.

Symptoms can include:

• Dry mouth. If you have Sjogren's syndrome, you produce far less saliva than normal. This makes chewing, swallowing and speaking difficult, and it may diminish your sense of taste as well as produce halitosis, or bad breath.

• Dry eyes. Your eyes may feel dry and gritty—they may also burn and turn red and be extremely sensitive to sunlight. A thick substance may accumulate in the inner corner of your eyes while you sleep. If not properly treated, Sjogren's syndrome can sometimes lead to ulcers of the cornea. On rare occasions, this can cause blindness.

• Swollen salivary glands. Two sets of major salivary glands produce saliva. They are under your tongue and in the cheeks in front of your ears. They may feel swollen and tender.

• Dental cavities. Saliva fights bacteria and defends against cavities. If you have Sjogren's syndrome, your saliva will be decreased, and your teeth may develop cavities more easily.

• Dry nose, throat and lungs. Your throat feels dry and tickly, and you may have a dry cough, hoarseness, a decreased sense of smell and nosebleeds. Sjogren's can also lead to pneumonia, bronchitis and ear problems.

• Vaginal dryness. Because Sjogren's can affect the mechanisms for secreting vaginal fluids, you may find sexual penetration difficult.

• Fatigue. Sjogren's may cause you to tire more easily or feel an underlying fatigue. Fatigue is also frequently a symptom of other autoimmune disorders, such as rheumatoid arthritis or lupus.

• Joint pain. If the immune system attack spreads to the joints, movement of fingers or other joints can become difficult or painful. Joint involvement may indicate you have another autoimmune disorder, such as rheumatoid arthritis or lupus.

• Other problems. Sjogren's syndrome can affect other parts of the body, such as blood vessels, the nervous system, muscles, skin and other organs. This can lead to muscle weakness, confusion and memory problems, dry skin and feelings of numbness and tingling. Sjogren's syndrome can also affect the liver and spleen. People with Sjogren's also have a 44 percent increased risk of developing lymphoma (cancer of the lymphocytes). Although this is unusual, it is another reason why medical exams and continued follow-up are critical.

With such a broad array of symptoms, in varying severity from patient to patient, diagnosis can be difficult. You and your health care professional may go through several steps before confirming the diagnosis of Sjogren's syndrome.

• Physical examination. Your health care professional will probably ask you to describe your symptoms. He or she will then look for other signs of Sjogren's, such as red, itchy eyes; swollen salivary glands; a dry, cracked tongue; and enlarged salivary glands in your neck. You'll also want to discuss what medications you are taking—both prescription and over-the-counter, because many medications have anticholinergic effects that may cause dryness of the mouth and eyes.

• Blood tests. Antinuclear antibodies (ANA), a group of antibodies that react against normal components of a cell nucleus, may be found in patients with Sjogren'ssyndrome, as well as the antibodies SSA (or RO) and SSB (or LA). However, not everyone with Sjogren's has these markers, and not everyone with these markers has Sjogren's syndrome.

• Schirmer test. This helps determine how dry your eyes are. It involves placing a small piece of filter paper under your lower eyelid to measure tear production.

• Rose Bengal and Lissamine Green. These dyes are used to detect epithelial defects on the surface of the eye.

• Lip biopsy. In this test, the health care professional removes a few minor salivary glands from inside your lower lip. The tissue is examined under a microscope. The appearance of the tissue helps determine if you have Sjogren's syndrome.

• Salivary function tests. The Parotid Gland Flow measures the amount of saliva you produce over a certain period of time. Salivary Scintigraphy also measures salivary gland function. Sialography is an x-ray of the salivary-duct system.

• Urine tests. These may be done to test your kidney function.

• Chest x-ray. This can help detect inflammation in the lungs.

Treatment

Treatment

There is no cure for Sjogren's syndrome, and no treatment has yet been found to restore physiological glandular secretions. This means that treatment primarily addresses the symptoms and is designed to relieve your discomfort and lessen the effects of dryness. Put another way, you can generally learn to manage your condition, but the root problems will remain. You may be referred to a rheumatologist for treatment.

Sjogren's syndrome can affect various parts of your body, so regular checkups can help detect and prevent future problems. And if you have secondary Sjogren's syndrome—that is, if the disorder is associated with another autoimmune condition—be sure to follow treatments for that condition.

Since Sjogren's syndrome affects everyone differently, your treatment plan will be based on your specific needs. But in general, moisture-replacement therapies may ease the symptoms of dryness. In some cases, your health care professional may recommend a simple procedure that blocks tear drainage from your eyes.

Dry eyes respond to the use of artificial tears applied every one to four hours or to pharmacological stimulation of tear production. Dry mouth can be relieved by drinking water, chewing sugar-free gum or using artificial saliva or saliva stimulants (such as pilocarpine and cevimeline). Wearing protective eyewear, such as goggles, or using a humidifier to keep moisture in the air can be helpful.

Nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin and ibuprofen, may be used to treat musculoskeletal symptoms. If you have severe complications, your health care professional may prescribe corticosteroids or immunosuppressive drugs.

Here's an overview of some of the most commonly used medications:

• NSAIDs can suppress inflammation and reduce pain. Sjogren's patients are generally prescribed NSAIDs for joint pain, muscle ache, fever and fatigue. However, NSAIDs provide no improvement in the salivary or tear flow.

• Corticosteroids may be used if you have internal organ involvement—which is rare—or as a topical treatment for dry eyes. If you have secondary Sjogren's syndrome, you may already be on corticosteroids—especially if the primary condition is lupus or rheumatoid arthritis. The side effects can be significant, so you and your health care professional need to monitor both the efficacy and possible side effects of the medication.

• Immunomodulating agents are occasionally used in autoimmune diseases like Sjogren's. These medications act very slowly, so they don't bring about immediate relief. As with corticosteroids, these immunomodulating agents, with the exception of hydroxychloroquine, are generally used when there is internal organ involvement. These agents can include:

  • Hydroxychloroquine (Plaquenil), an antimalarial drug, is sometime used to relieve swelling, stiffness and pain. It may also have a limited effect on the glandular symptoms, the hallmark of the disease.

  • Methotrexate (Trexall) is a folic-acid inhibitor generally used to treat rheumatoid arthritis and may be prescribed for Sjogren's patients with internal organ involvement.

  • Cyclophosphamide (Cytoxan) is considerably more potent than methotrexate and is reserved for the most serious organ involvement. Other drugs used for severe organ involvement include mycophenolate (CellCept) and azathioprine (Azasan).

  • Antifungal drugs, such as nystatin (Bio-Statin, Nystop) may be used to treat oral candidiasis (a common fungal infection).

  • Pilocarpine (Salagen) and cevimeline (Evoxac) tablets may be use to increase salivary flow. Evoxac, however, should not be used for those with a number of other conditions, including asthma and acute angle glaucoma. Higher doses of both of these medications may have significant side effects including flushing, sweating, nausea, vomiting and diarrhea.

• Saliva substitutes can be used to replace oral secretions, but they have not been carefully studied, and they are only recommended when saliva secretions cannot be replaced by sips of water.

• Artificial tears or eye drops can help relieve the discomfort of dry eyes. You probably want to use preservative-free products, especially if you apply the drops frequently. These products include Tears Naturale Free and Bion Tears. (Note: Some over-the-counter eye drops contain vasoconstrictors and can cause further drying.)

• Cyclosporine A (Restasis) eye drops are immunosuppressive and are also used to increase tear production. Unlike artificial tears, these are available only by prescription.

As for the future, a variety of novel therapeutic approaches, using newer immunosuppressive agents, drops made from a person's own blood serum, and the injected medication rituximab (Rituxan) are being investigated but are not ready for widespread clinical application.

Prevention

Prevention

Sadly, there's no known way to prevent the onset of Sjogren's syndrome. However, the symptoms are often treatable, and you can develop strategies for keeping some symptoms at bay.

Ironically, the best "prevention" is early diagnosis and prevention of complications. Early diagnosisand intervention can impede the course of Sjogren's syndrome. All too often, the condition goes undiagnosed and symptoms continue to worsen. Since Sjogren's syndrome can affect many parts of the body, regular checkups can help detect and prevent future problems. You and your health care professional should meet frequently to discuss your treatment strategy to keep the condition from worsening.

A healthy diet is part of taking care of yourself under any circumstances, and it's doubly important if you have Sjogren's. You should probably avoid alcoholic and caffeinated beverages as they may be dehydrating. Spicy and acidic food can irritate your mouth, and sugary food can promote tooth decay.

Of course, get plenty of rest and avoid tobacco and (when possible) stress. Mild exercise, such as walking or swimming, can help keep joints and muscles flexible. Exercise may also protect against further joint damage. Warm compresses or heating pads can help ease joint or gland pain.

Keeping your mouth moist is important. Artificial moisture can help. Methylcellulose swabs or spray might help alleviate mouth dryness. Sipping fluids throughout the day may help, too, as well as sugar-free gum or candies, which stimulate saliva production. Use artificial tears or eye drops to relieve the discomfort of dry eyes. Medications such as Salagen and Evoxac may improve saliva production, and Restasis eye drops may improve tear production.

Practicing good oral hygiene is essential. Frequent dental checkups, fluoride and mouth rinses, regular brushing with antibacterial toothpastes and flossing are more important for Sjogren's patients, since you are at a higher risk for tooth decay.

Here are other coping strategies that can help moderate your symptoms.

• Don't smoke; avoid tobacco smoke and other air irritants.

• Use self-adhesive stamps and envelopes.

• Don't direct hair dryers toward your eyes.

• Wear glasses on windy days and goggles when swimming.

• Coat your lips with petroleum-based lubricants to prevent drying. (Many lipsticks can provide this protection).

• Keep your home humidity between 30 percent and 50 percent. You may want to use a humidifier year-round. Some experts advise using a cool-mist, ultrasonic humidifier; be sure to clean it daily.

• Avoid rubbing your eyes.

• If you have nasal and airway dryness, consider using a soft cervical collar while you sleep to help prevent your mouth from opening, thus preventing the dryness that mouth breathing causes.

• Avoid abrasive detergents, soaps, and in some cases, dryer sheets.

• Consider soft contact lenses rather than the traditional hard ones. Many patients with Sjogren's are unable to wear contact lenses.

• Avoid prolonged hot showers or baths.

• Talk to your health care professional about discontinuing your use of decongestants and antihistamines because they dry your mouth and nasal areas.

• Use sunscreen.

• Apply lotions or other lubricants to still-damp skin right after you finish your bath or shower.

• Frequent small sips of water—or sucking on ice chips—can help keep your lips and gastrointestinal tract hydrated.

• Avoid drafts from air conditioners, heaters and radiators, when possible.

• If you have vaginal dryness, use lubricants made specifically for that purpose.

Finally, experts stress the importance of mental and emotional health. Joining a self-help or support group can help you cope emotionally, as well as teach you new strategies for managing your disease.

Facts to Know

Facts to Know

1. Women are nine times more likely than men to develop Sjogren's (pronounced "show-grins") syndrome, a chronic, incurable disorder in which immune system cells attack and destroy the glands that produce tears and saliva. Sjogren's can occur at any age, but women are at higher risk after menopause.

2. The hallmark symptoms of Sjogren's are dry eyes and dry mouth. The disorder may also cause skin, nose and vaginal dryness, and, in rare cases, affect other organs of the body as well, including your kidneys, blood vessels, lungs, liver, pancreas and brain.

3. The Sjogren's Syndrome Foundation estimates that four million Americans suffer from this disorder; many go undiagnosed.

4. The syndrome, named after Swedish ophthalmologist Henrik Sjogren, is an autoimmune disorder, which means that your immune system attacks your body's healthy tissues. Ordinarily, the immune system produces antibodies that target such destructive material as viruses and bacteria. In the case of Sjogren's and other autoimmune diseases, autoantibodies—antibodies turned against the self—are produced.

5. Sjogren's syndrome may occur alone (primary Sjogren's syndrome), or it may be a symptom of other autoimmune/rheumatic diseases, such as rheumatoid arthritis, lupus, polymyositis and some forms of scleroderma (secondary Sjogren's syndrome).

6. A blood marker often found in women with Sjogren's syndrome can, very rarely, be associated with heart problems or neonatal lupus in newborn babies. If you have Sjogren's syndrome and plan to become pregnant, see your health care professional about testing for this marker and deciding what to do if the marker is present.

7. There is no cure for Sjogren's syndrome, and no treatment has yet been found to restore total glandular secretions. This means that treatment addresses the symptoms only, and is designed to relieve your discomfort and lessen the effects of dryness. Put another way, you can generally learn to manage your condition, but the root problems will remain.

8. Much about Sjogren's is still unknown. As yet, no single gene, microorganism or hormone has been implicated as a cause of Sjogren's syndrome. Sadly, there's no way to prevent the onset of Sjogren's syndrome. However, the symptoms are often treatable, and you can develop strategies for keeping some symptoms at bay.

9. A healthy diet is part of taking care of yourself under any circumstances, and it's doubly important if you have Sjogren's. You should probably avoid alcoholic and caffeinated beverages because they are dehydrating. Spicy and acidic food can also irritate your mouth, and sugary food can promote tooth decay.

10. Since Sjogren's syndrome affects everyone differently, your treatment plan will be based on your specific needs. In general, moisture-replacement therapies may ease the symptoms of dryness. In some cases, your health care professional may recommend a simple operation that blocks tear drainage from your eye.

Questions to Ask

Questions to Ask

Review the following Questions to Ask about Sjogren's syndrome so you're prepared to discuss this important health issue with your health care professional.

1. Do my symptoms suggest Sjogren's syndrome?

2. Does it appear likely that I have secondary Sjogren's syndrome and need to seek treatment for another autoimmune disorder?

3. What are the side effects of the drugs being prescribed for me? Is there a possibility of adverse interactions with other medications I am taking?

4. How will I know if the disease is progressing? How can I identify potential complications or new symptoms early?

5. What should I do if additional symptoms develop?

6. What are some good non-medical approaches to managing Sjogren's?

7. Will Sjogren's affect my ability to become pregnant?

8. What changes should I make in oral hygiene to protect against cavities?

9. How can I best protect my eyes?

10. Will this disorder affect other health conditions I have?

Key Q&A

Key Q&A

1. Who gets Sjogren's syndrome? Am I at risk?

   Women are nine times more likely to develop Sjogren's syndrome than men, and the disorder occurs sometimes in connection with another autoimmune disease, such as rheumatoid arthritis, lupus or scleroderma. Women are athigher risk for developing Sjogren's after menopause. Other than those, there are no clear risk factors for predicting who will get Sjogren's.

2. How can I prevent the disease?

   Researchers have not found any special dietary guidelines or lifestyle modifications that can stave off the onset of Sjogren's. The best that can be done is to diagnose the disease as early as possible and start aggressive treatment to try to prevent worsening of symptoms.

3. Why is Sjogren's called an autoimmune disease?

   Ordinarily, the immune system produces antibodies that target such destructive material as viruses and bacteria. In the case of Sjogren's and other autoimmune diseases, autoantibodies-antibodies that turn against the part of the body they should protect—are produced. With Sjogren's, immune-system cells attack and destroy the glands that produce tears and saliva.

4. What are the symptoms of Sjogren's?

   The hallmark symptoms of Sjogren's are dry eyes and dry mouth. The disorder may also include skin, nose and vaginal dryness, as well as swollen salivary glands, joint pain and fatigue.

5. I have dry mouth and dry eyes. Do I really need to see a health care professional, or can I just use artificial tears and sip water?

   Early diagnosis and a systematic treatment plan are essential for slowing the disease's progress. Sjogren's syndrome can affect other parts of the body, such as blood vessels, the nervous system, muscles, skin and other organs. This can lead to muscle weakness, confusion and memory problems, dry skin, and feelings of numbness and tingling. Sjogren's sy also affect the liver and pancreas. When it does, there is a greater chance for developing cancer of the lymph tissue. Although this is unusual, it is another reason why medical exams and continued follow-up are critical.

6. How is Sjogren's syndrome diagnosed?

   Once Sjogren's is suspected, blood tests for autoantibodies, tests to determine the degree of dry eye and mouth and sometimes lip biopsies can point to the presence of Sjogren's.

7. Is it safe to get pregnant if I have Sjogren's?

   A blood marker often found in women with Sjogren's syndrome can, very rarely, be associated with heart problems or neonatal lupus in newborn babies. If you have Sjogren's syndrome and plan to become pregnant, see your health care professional about testing for this marker and deciding what to do if the marker is present.

8. What treatments are available for Sjogren's?

   Artificial tears (such as Tears Naturale Free and Bion Tears) combat dry eyes, and saliva substitutes (such as Glandosane, Moi-Stir, and Salivart) combat dry mouth. Saliva substitutes have not been carefully studied, however, and they are only recommended when saliva secretions cannot be replaced by sips of water. Talk to your health care professional before using any of these products. Cyclosporine A (Restasis) eye drops are immunosuppressive and are also used to increase tear production. Unlike artificial tears, these are available only by prescription. Over-the-counter lubricating products are also available to address vaginal, skin and nasal dryness. Pilocarpine (Salagen) tablets may be prescribed to increase salivary flow. If symptoms include joint pain, muscle ache, fever and fatigue, nonsteroidal anti-inflammatory drugs such as aspirin or ibuprofen may be prescribed or recommended. In a case of internal organ involvement, your health care professional may recommend more aggressive treatments, such as corticosteroids or immunomodulating agents.