Sjögren's

Overview

What Is It?

Sjögren's syndrome is an autoimmune disorder in which the glands that produce moisture are destroyed. The hallmark symptoms are dry eyes and dry mouth.

Women are nine times more likely than men to develop Sjögren's, a chronic, disorder in which immune system cells can attack and destroy the glands that produce moisture, causing effects throughout the body. Sjögren's (pronounced "SHOW-grins") can occur at any age, but the average age for diagnosis is 40 years old, according to a recent survey by the Sjögren's Syndrome Foundation.

The foundation estimates that about 4 million Americans suffer from this Sjögren's, with an estimated 2.5 million people currently undiagnosed.

The disease, named after Swedish ophthalmologist Henrik Sjögren, is an autoimmune disorder––a disorder in which your immune system attacks your 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 Sjögren's and other autoimmune diseases, the immune system produces autoreactive cells and autoantibodies, which turn against various parts of the body.

Sjögren's may occur alone (referred to as primary Sjögren's), or it may be associated with other autoimmune/rheumatic diseases, such as rheumatoid arthritis, lupus, polymyositis, scleroderma and primary biliary cholangitis. When Sjögren's occurs with another autoimmune disease it is called secondary Sjögren's. The Sjogren's symptoms may be equally severe with either type.

The hallmark symptoms of Sjögren's are dry eyes and dry mouth. However, the disorder may also cause skin, nose, upper airway, 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. Your eyes may also be more sensitive to light (photosensitivity).

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. Severe tooth decay is an important clue to diagnosing Sjögren's.

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.

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; permit proper swallowing of food; and contain digestive enzymes that begin breaking down food. Saliva also facilitates your ability to taste and to speak. All of these functions can be affected by Sjögren's.

Salivary glands are a prime target of Sjögren'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 Sjögren's, autoreactive lymphocytes (a type of white blood cell) infiltrate and may destroy the glandular tissue and affect gland function.

In severe cases, Sjögren's can damage vital organs. 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 can 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, memory changes and joint pain. Also, there is a significantly elevated risk of lymphoma in people with Sjögren's compared to the general population.

An autoantibody to the protein SS-A/Ro, found in women with Sjögren's, can very rarely be associated with congenital heart block and neonatal lupus in newborn babies. If you have Sjögren's 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

No single gene, microorganism or hormone is known to cause Sjögren's. As a result, diagnosis is often difficult. Its symptoms can mimic those of other diseases, and no two people with Sjögren's 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, dry eyes to an optometrist or nerve pain to a neurologist—so the condition often goes undiagnosed. In fact, the average time from the onset of Sjögren's symptoms to diagnosis is three years.

Once Sjögren'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 salivary gland biopsies, can point to the presence of Sjögren's.

Symptoms can include:

• Dry mouth. If you have Sjögren's syndrome, you can 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. The decrease in saliva can also lead to yeast overgrowth (candidiasis/thrush), which can cause painful red, cracks at the corners of your lips.
• 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. In addition, your eyelids may become inflamed, and the oil-producing glands in the eyelids can become blocked. If not properly treated, Sjögren's can sometimes lead to ulcers of the cornea. On rare occasions, this can cause loss of vision.
• Swollen salivary glands. Three sets of major salivary glands produce saliva. They are under your tongue, under your lower jaw 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 Sjögren's, your saliva may be decreased, and your teeth may develop cavities more easily. The cavities can appear at your gum line, an indicator of Sjögren's.
• 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. Sjögren's can also lead to pneumonia, bronchitis, ear problems and gastroesophageal reflux.
• Vaginal dryness. Because Sjögren's can affect the mechanisms for secreting vaginal fluids, you may find sexual penetration painful.
• Fatigue. Sjögren's may cause you to tire more easily or feel an underlying, profound fatigue. Fatigue is frequently a symptom of other autoimmune disorders, such as rheumatoid arthritis or lupus, however it is particularly severe in Sjögren's. (Find out more about the "13 Types of Sjogren's Fatigue.")
• 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.
• Digestive problems. Abnormal functioning of the gastrointestinal system including esophagus, stomach, small and large intestine (colon).
• Other problems. Sjögren's 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. Sjögren's can also affect the liver, sometimes related to autoimmune hepatitis or autoimmune cholangitis, and, in rare instances, may affect the spleen. People with Sjögren's also have an increased risk of developing lymphoma (cancer of the lymphocytes) compared to the general population. Although this is unusual, it is another reason 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 Sjögren's.

• Medical history and physical examination. Your health care professional will probably ask you to describe your symptoms. He or she will then look for other signs of Sjögren's, such as red, itchy eyes; swollen salivary glands or lymph nodes; and a dry, cracked tongue. You'll also want to discuss what medications you are taking—both prescription and over-the-counter, because many medications can cause dryness of the mouth and eyes.
• Blood tests. Antinuclear antibodies, a group of autoantibodies that react against normal components of a cell nucleus, may be found in people with Sjögren's, as well as the antibodies SS-A (or Ro) and/or SS-B (or La). However, not everyone with Sjögren's has these markers, and not everyone with these markers has Sjögren's. Newer biomarkers have been identified, including autoantibodies to salivary protein-1, carbonic anhydrase-6 and parotid specific protein. These markers may appear years before the SS-A/SS-B antibodies. A diagnostic test kit (Sjö) checks for these newer biomarkers, along with the classic ones, and may lead to an earlier diagnosis. A complete blood count may also show anemia or low white blood count, which could indicate leukopenia, another clue for diagnosis of Sjögren's.
• 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.
• Lissamine green, rose bengal and sodium fluorescein. These dyes are used to detect dry spots on the surface of the eye. Lissamine green is least irritating to the eye and is the preferred dye.
• Salivary gland biopsy. In this test, the health care professional removes a few minor salivary glands, usually from inside your lower lip. The tissue is examined under a microscope. The appearance of the tissue helps determine if you have Sjögren's.
• Salivary function tests. To measure the amount of saliva you produce over a certain time, your health care professional may order tests such as a salivary scintigraphy, which involves injection of a radioactive isotope, or a sialography,an x-ray of the salivary-duct system that shows how much saliva flows into your mouth. He or she may also order a spit test, during which you spit into a test tube every minute for 15 minutes, to measure your saliva production.
• Urine tests. These may be done to test your kidney function.
• Chest x-ray. This can help detect inflammation in the lungs.

Treatment

There is no cure for Sjögren's, and no treatment has yet been found to restore normal physiological glandular secretion. Secretagogues can restore glandular function, but only as long as you're on the medication. Rituxima (Rituxan) can restore function in some people, but the benefit doesn't last, and the drug may only be used in limited types of cases.

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 may remain. You may be referred to a rheumatologist for treatment.

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

Since Sjögren's 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 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 and/or immunosuppressive drugs.

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

• NSAIDs can suppress inflammation and reduce pain. Sjögren'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. If you have secondary Sjögren's, 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 effectiveness and possible side effects of the medication.
• Hydroxychloroquine (Plaquenil), an antimalarial drug, is often used to relieve swelling, stiffness and pain. It may also have a limited effect on the glandular symptoms, a hallmark of the disease.
• Immunomodulating agents are occasionally used in autoimmune diseases like Sjögren's. These medications can act slowly, so they don't bring about immediate relief. As with corticosteroids, these immunomodulating agents are generally used when there is internal organ involvement. They also may be used to allow the tapering or stopping of steroids. These agents can include:
  • Methotrexate (Trexall) is a folic-acid inhibitor generally used to treat rheumatoid arthritis and may be prescribed in rare cases for Sjögren's patients with internal organ involvement. It can be a good choice for people who have Sjögren's with severe arthritis.
  • 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, Imuran).
  • Rituximab (Rituxan) is a biologic monoclonal antibody that depletes certain B-cell lymphocytes. It is given by intravenous infusion and effects may last six months.
• Antifungal drugs, such as nystatin (Bio-Statin) may be used to treat oral candidiasis (a common fungal infection).
• Pilocarpine (Salagen) and cevimeline (Evoxac) tablets may be used 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, among others.
• Saliva substitutes such as Oasis Mouth Moisturizing Spray, Mouth Kote spray, Biotene mouth spray and gel, and coconut oil can be used to replace oral secretions.
• Artificial tears or eyedrops can help relieve the discomfort of dry eyes. You 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 eyedrops contain vasoconstrictors and can cause further drying.)
• Cyclosporine A (Restasis) eyedrops decrease inflammation and are also used to increase tear production. Unlike artificial tears, these are available only by prescription.
• Lifitegrast (Xiidra) eyedrops also decrease inflammation, but by a different mechanism and are newly available by prescription.
• Hydroxypropyl cellulose (Lacrisert), a small dissolvable cellulose pellet that fits into your lower eyelid, may also be helpful in the treatment of dry eyes. Lacrisert is also only available by prescription.

Prevention

There's no definite way to prevent the onset of Sjögren's. However, the symptoms are often treatable, and you can develop strategies for keeping some symptoms at bay.

Early diagnosis and intervention may slow the progress of Sjögren's and prevent complications. In the early stages, you may find some relief through diet changes and stress management, as well as medications.

All too often, the condition goes undiagnosed and symptoms continue to worsen. Since Sjögren's can affect many parts of the body, regular checkups can help detect and prevent 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 Sjögren's. You should probably avoid alcoholic and caffeinated beverages because they may be dehydrating. Spicy and acidic food can irritate your mouth, and sugary food can promote tooth decay and inflammation.

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 and massage can help ease joint or gland pain.

Keeping your mouth moist is important. Artificial moisture can help. Methylcellulose swabs or spray may help alleviate mouth dryness and lower your risk of ulcers. Sipping sugar-free fluids throughout the day may help, too, as well as sugar-free gum or candies, which stimulate saliva production. Use artificial tears or eyedrops to relieve the discomfort of dry eyes. Medications such as Salagen and Evoxac may improve saliva production, and eyedrops containing cyclosporine A (Restasis) 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 Sjögren'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 also provide this protection.)
• 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 Sjögren'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 bathe 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

1. Women are nine times more likely than men to develop Sjögren's (pronounced "show-grins"), a chronic disorder in which immune system cells attack and destroy the glands that produce tears and saliva. Sjögren's can occur at any age, but women are at higher risk with increasing age.
2. The hallmark symptoms of Sjögren's are dry eyes and dry mouth, but these may not be major symptoms in all cases. The disorder may also cause nose and vaginal dryness and dry, itchy skin. In rare cases, it may affect other organs of the body as well, including your kidneys, blood vessels, lungs, liver, pancreas and brain.
3. The Sjögren's Syndrome Foundation estimates that four million Americans suffer from this disorder; an estimated 2.5 million go undiagnosed.
4. The syndrome, named after Swedish ophthalmologist Henrik Sjögren, 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 Sjögren's and other autoimmune diseases, autoantibodies—antibodies turned against the self—are produced.
5. Sjögren's may occur alone (primary Sjögren's), or it may occur with another autoimmune/rheumatic diseases, such as rheumatoid arthritis, lupus, polymyositis, primary biliary cholangitis and some forms of scleroderma (secondary Sjögren's).
6. A blood marker often found in women with Sjögren's can, very rarely, be associated with heart problems or neonatal lupus in newborn babies. If you have Sjögren's 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 Sjögren's, and no treatment has yet been found to permanently restore total glandular secretions. This means that treatment primarily 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 may remain.
8. Much about Sjögren's is still unknown. Certain genes put people at a higher risk for the disorder, but scientists think some sort of a trigger—such as a virus or bacteria—is also necessary to bring it on. Unfortunately, there's no way to prevent the onset of Sjögren'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 Sjögren'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 and inflammation.
10. Because Sjögren's 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

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

1. Do my symptoms suggest Sjögren's?
2. Does it appear likely that I have secondary Sjögren's 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 nonmedical approaches to managing Sjögren's?
7. Will Sjögren's affect my ability to become pregnant?
8. How can I protect against cavities?
9. How can I best protect my eyes?
10. Will this disorder affect other health conditions I have?

Key Q&A

Organizations and Support

For information and support on coping with Sjögren's Syndrome, please see the recommended organizations, books and Spanish-language resources listed below.

American Academy of Dermatology
Website: https://www.aad.org
Address: P.O. Box 4014
Schaumburg, IL 60618
Hotline: 1-866-503-SKIN (7546)
Phone: 847-240-1280
Email: mrc@aad.org

American Autoimmune Related Diseases Association (AARDA)
Website: https://www.aarda.org
Address: 22100 Gratiot Avenue
East Detroit, MI 48021
Hotline: 1-800-598-4668
Phone: 586-776-3900
Email: aarda@aarda.org

American College of Rheumatology
Website: https://www.rheumatology.org
Address: 1800 Century Place, Suite 250
Atlanta, GA 30345
Phone: 404-633-3777

National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Website: https://www.niams.nih.gov
Address: National Institutes of Health
Bldg. 31, Room 4C02 31 Center Dr. - MSC 2350
Bethesda, MD 20892
Hotline: 1-877-22-NIAMS (1-877-226-4267)
Phone: 301-496-8190
Email: niamsinfo@mail.nih.gov

National Institute of Neurological Disorders and Stroke
Website: https://www.ninds.nih.gov
Address: NIH Neurological Institute
P.O. Box 5801
Bethesda, MD 20824
Hotline: 1-800-352-9424
Phone: 301-496-5751

Sjögren's Syndrome Foundation
Website: https://www.sjogrens.com
Address: 6707 Democracy Blvd., Suite 325
Bethesda, MD 20817
Hotline: 1-800-475-6473
Phone: 301-530-4420
Email: tms@sjogrens.org

Books

New Sjogren's Syndrome Handbook
by Daniel J. Wallace

The Sjögren's Syndrome Survival Guide
by Teri P., Ph.D. Rumpf and Katherine Morland Hammitt

Spanish-language resources

Medline Plus: Sjogren's Syndrome
Website: https://www.nlm.nih.gov/medlineplus/spanish/sjogrenssyndrome.html
Address: US National Library of Medicine
8600 Rockville Pike
Bethesda, MD 20894
Email: custserv@nlm.nih.gov

National Institute of Arthritis and Musculoskeletal and Skin Diseases
Website: https://www.niams.nih.gov/Portal_en_espanol/Informacion_de_salud/Sindrome_de_Sjogren/default.asp
Address: National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) / National Intitutes of Health
1 AMS Circle
Bethesda, MD 20892
Hotline: 1-877-226-4267
Email: NIAMSinfo@mail.nih.gov