What is Sjögren's Syndrome?

March 2, 2010

Sjögren's syndrome (pronounced SHOW-grins) is a chronic autoimmune disease in which people's white blood cells attack their moisture-producing glands. Today, as many as four million Americans are living with this disease.

Table of Contents

• About Sjögren's
• Symptoms
• Cause & Effects

• Diagnosis
• Treatments
• Coping Tips

About Sjögren's Syndrome

Although the hallmark symptoms are dry eyes and dry mouth, Sjögren's may also cause dysfunction of other organs such as the kidneys, gastrointestinal system, blood vessels, lungs, liver, pancreas, and the central nervous system. Patients may also experience extreme fatigue and joint pain and have a higher risk of developing lymphoma.

With upwards of 4,000,000 Americans suffering from Sjögren's syndrome, it is one of the most prevalent autoimmune disorders. Nine out of 10 patients are women.

About half of the time Sjögren's syndrome occurs alone, and the other half it occurs in the presence of another autoimmune connective tissue disease such as rheumatoid arthritis, lupus, or scleroderma. When Sjögren's occurs alone, it is referred to as "Primary Sjögren's." When it occurs with another connective tissue disease, it is referred to as "Secondary Sjögren's."

All instances of Sjögren's syndrome are systemic, affecting the entire body. Symptoms may remain steady, worsen, or, uncommonly, go into remission. While some people experience mild discomfort, others suffer debilitating symptoms that greatly impair their functioning. Early diagnosis and proper treatment are important - they may prevent serious complications and greatly improve a patient's quality of life.

Since symptoms of Sjögren's syndrome mimic other conditions and diseases, Sjögren's can often be overlooked or misdiagnosed. On average, it takes nearly seven years to receive a diagnosis of Sjögren's syndrome. Patients need to remember to be pro-active in talking with their physicians and dentists about their symptoms and potential treatment options.

Since the disease was first identified in 1933 by Dr. Henrik Sjögren, it has been proven to affect virtually every racial and ethnic group. General awareness about Sjögren's syndrome is still lacking and increased professional awareness is needed to help expedite new diagnoses and treatment options.

Sjögren's syndrome affects everyone differently. You may not have every symptom listed here, and you may have only minor problems with those you do have. The symptoms may seem worse at some times than at others.

In people with no other health problems, the most common early symptom is the onset of severe dry mouth and eyes.

In people with rheumatoid arthritis or related conditions, dry eyes and mouth develop more slowly. In this case, Sjögren's may be difficult to diagnose.

Dry Mouth

The mouth normally contains saliva, which aids chewing and swallowing. In people with Sjögren's syndrome, the amount of saliva is much less. This makes chewing, swallowing, and speaking difficult. It may also cause a decreased sense of taste.

Dry Eyes

Your eyes may feel dry, "gritty," or "sandy." They may burn and look red. A thick substance may accumulate in the inner corner of your eyes while you sleep. Your eyes may be more sensitive to sunlight. If not properly treated, Sjögren's syndrome can lead to ulcers of the cornea (the clear covering of the eyeball). On rare occasions, this can cause blindness.

Swollen Salivary Glands

There are three set of glands that produce saliva. They're located under your tongue, in the cheeks in front of your ears, and in the back of your mouth. They may feel swollen and tender. This may occur along with a fever. This affects about one-half of people with the disorder.

Dental Cavities

This is a common problem that results from a dry mouth. Saliva fights bacteria and defends against cavities. Because you have decreased saliva, your teeth may develop cavities more easily.

Dry Nose, Throat, and Lungs

This may make your throat feel dry and tickly. You may have a dry cough, hoarseness, a decreased sense of smell, and nosebleeds. It can also lead to pneumonia, bronchitis, and ear problems.

Dryness of the Vagina

This can cause painful intercourse for women with Sjögren's syndrome.

Fatigue

Fatigue is a common complaint. You may get easily exhausted and feel tired and worn out.

Other Problems

Sjögren'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.

Sjögren's syndrome can 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 one reason why medical exams and continued follow-up are important.

While the exact cause of Sjögren's syndrome is not known, there is growing scientific support for genetic (inherited) factors. The illness is sometimes found in other family members. It is also found more commonly in families that have members with other autoimmune illnesses, such as systemic lupus erythematosus, autoimmune thyroid disease, juvenile diabetes, etc. About 90% of patients with Sjögren's syndrome are female.

In Sjögren's syndrome, changes occur in the immune system - the body's defense against disease. In Sjögren's, the immune system lacks the usual controls. This causes white blood cells to invade glands in the body that produce moisture, such as the tear and salivary glands, and the Bartholins glands in the vagina. They can destroy the glands and cause them to stop producing moisture.

Sjögren's syndrome can also cause problems in other parts of the body, including the joints, lungs, muscles, kidneys, nerves, thyroid gland, liver, pancreas, stomach, and brain. In addition, Sjögren's syndrome may cause skin, nose, and vaginal dryness, and may affect other organs of the body including the kidneys, blood vessels, and lungs. Blindness is a rare complication of Sjögren's.

Early diagnosis and treatment are important for preventing complications. Unfortunately, reaching a diagnosis can often be difficult and has been found to take an average of 6.5 years from the onset of symptoms.

Sjögren's syndrome symptoms frequently overlap with or "mimic" those of other diseases including lupus, rheumatoid arthritis, fibromyalgia, chronic fatigue syndrome, and multiple sclerosis. Dryness can also occur for other reasons, such as a side effect of medications such as anti-depressants and high blood pressure medication.

There is no single test that will confirm diagnosis. Rheumatologists have primary responsibility for diagnosing and managing Sjögren's syndrome and can conduct a series of tests and ask about symptoms. An international group of experts formulated classification criteria for Sjögren's syndrome which help doctors arrive at a diagnosis. These criteria consider dryness symptoms, changes in salivary (mouth) and lacrimal (eye) gland function, and systemic (whole body) findings.

Blood tests your physician may perform include:

• ANA (Anti-Nuclear Antibody)
  ANAs are a group of antibodies that react against normal components of a cell nucleus. About 70% of Sjögren's patients have a positive ANA test result.


• (Rheumatoid Factor)
  This antibody test is indicative of a most often performed for the diagnosis of rheumatoid arthritis (RA) but is positive in many rheumatic diseases. In Sjögren's patients, 60-70% have a positive RF.


• SS-A (or Ro) and SS-B (or La)
  These are the marker antibodies for Sjögren's. Seventy percent of Sjögren's patients are positive for SS-A and 40% are positive for SS-B (these may also found in lupus patients)


• ESR (Erythrocyte Sedimentation Rate)
  This test measures inflammation. An elevated ESR indicates the presence of an inflammatory disorder, including Sjögren's syndrome.


• IGs (Immunoglobulins)
  These are normal blood proteins that participate in immune reactions and are usually elevated in Sjögren's patients.

The Ophthalmologic (eye) Tests Include:

• Schirmer Test
  Measures tear production.


• Rose Bengal and Lissamine Green
  Eyedrops containing dyes that an eye care specialist uses to examine the surface of the eye for dry spots.

The Dental Tests Include:

• Salivary Flow
  Measures the amount of saliva produced over a certain period of time.


• Salivary scintigraphy
  A nuclear medicine test that measures salivary gland function.


• Salivary gland biopsy (usually in the lower lip)
  Confirms inflammatory cell (lymphocytic) infiltration of the minor salivary glands.

Your physician will consider the results of these tests along with your physical examination to arrive at a final diagnosis. Further research is being conducted to refine the diagnostic criteria for Sjögren's syndrome and to help make diagnosis easier and more accurate.

Early diagnosis and high-quality professional care are extremely important for Sjögren's patients.

Currently, there is no cure for Sjögren's syndrome. However, treatments may improve various symptoms and prevent complications.

In addition to over the counter (OTC) eye drops and mouth preparations, prescription products for dry eyes and dry mouth are available. They include:

For Dry Mouth:

• Evoxac® (cevimeline)
• Salagen® (pilocarpine hydrochloride)
• Numoisyn™

For Dry Eye:

• Restasis® (cyclosporine ophthalmic emulsion)
• Lacrisert® (hydroxypropyl cellulose ophthalmic insert)

Some patients are prescribed immunosuppressive medications to treat their internal organ manifestations. Physicians may also prescribe other medications for systemic manifestations or severe flares. Since Sjögren's syndrome affects each patient differently, a personalized plan should be developed by you and your physician, dentist and eye care specialist about how to treat your various symptoms.

In addition, many symptoms and problems of Sjögren's syndrome can be treated with over-the-counter medications. These medications can help to alleviate different types of dryness and pain, but you should check with your physician when adding these medications to your regimen. Your doctor may have suggestions for what products you should use and may also give you some tips on how and when to use them.

Look below through the categories for over 100 short but essential survival tips for Sjögren's patients.

General Sjögren's Coping Tips

• It's important to find a doctor who is a good partner in treating your disease, and a good listener!


• When seeking information on Sjögren's, consider the source. Make sure the source is authoritative, knowledgeable and up-to-date.


• Identify major stressors in your life and work with a mental health professional or your support system to minimize their impact.


• Just because an OTC product is natural does not make it safe or appropriate. Always discuss natural remedies with your doctor.


• Recent scientific data show that longevity is associated with the successful management of chronic diseases such as Sjögren's syndrome - not the absence of any disease.


• If you have Sjögren's and are employed, ask for needed accommodations because of your medical condition.


• Always take non-steroidal anti-inflammatory drugs (NSAIDs) with food or milk to avoid stomach upset.


• Never feel guilty about seeking a second opinion, especially given the overlap and uncertainty with Sjögren's.


• "No one grew up with plans to have a chronic disease. It just happens. Once it does, you have to do everything possible to live in the best way you can." (From The Sjögren's Syndrome Survival Guide.)


• If you are riding an emotional roller coaster of emotions because of your Sjögren's, join an SSF Support Group and/or talk to a health professional. You will feel better knowing that you are not alone, plus you will find more ways to cope with your disease.


• Listen to your body. Do not take on more than you can handle, and pay attention to new symptoms, or symptoms that get worse, and tell your physician.


• Find a doctor who will coordinate all of your care in Sjögren's and head your "medical care team." Usually this is a rheumatologist, but a family doctor or general practitioner may also fill this role.


• Become an active participant in treatment decisions and an informed consumer. You will feel more in control of your disease, decrease anxiety, and ensure you are getting the best possible medical care for you.


• You can maintain a good quality of life with Sjögren's. Stay on top of the latest information, educate yourself through the SSF, find support and watch your limits.


• Make sure your physician knows about all the prescription and OTC medications you are taking. Many drugs have side effects that can make your Sjögren's symptoms worse.


• Remember that just because a symptom can't be seen easily, your symptom is still important. If you feel your Sjögren's symptoms are dismissed by a physician, help educate your physician and/or find another physician.


• Wondering what products Sjögren's patients use for dry eye, dry mouth, dry nose, dry skin, dry ears, dry vagina, sun protection, reflux and neuropathy pain? Contact the SSF for a copy of its Product Directory.


• Seek positive relationships in your life. These will help you cope and will reduce general anxiety when you have a frequently misunderstood disease.

Dry Mouth Coping Tips

• Eat soft, moist foods if you have trouble swallowing or with your teeth chipping and breaking.


• Sjögren's patients should eat smaller, more frequent meals to stimulate saliva flow.


• Avoid salty, acidic or spicy foods and carbonated drinks that may be painful on your dry mouth or interfere with digestion in Sjögren's.


• Help prevent dental decay by using oral products containing the sweetner xylitol.


• For dry mouth, increase your intake of liquids during the day. Remember that small sips of water work best.


• Sjögren's patients should avoid mouthwashes and rinses that contain alcohol or witch hazel. These ingredients can aggravate oral dryness and burning.


• Chew sugar-free gum or suck on hard diabetic or sugar-free candies to help increase saliva.


• Apply vitamin E oil or moisturizing gels to dry or sore parts of the mouth or tongue for long-lasting relief. Use the liquid oil or punch holes in vitamin capsules.

Dry Eye Coping Tips

• Try sterile eyelid cleansers or baby shampoo on a warm washcloth to help with blepharitis, a common condition in Sjögren's that causes chronic inflammation of the eyelids and eyelid margins.


• The mainstay of treatment for blepharitis, a chronic condition that accompanies dry eye and Sjögren's, is warm compresses, lid massage and lid hygiene. If the blepharitis is acute, you might need a prescription antibiotic ointment.


• If your eyes are bothered by light, wear sunglasses or try lenses with a FL-41 filter.


• Carry a wet washcloth in a zip-top bag to place on your dry eyes when traveling.


• Avoid applying anything to the eyelids that can irritate your dry eye; products placed on the eyelid will get into the tear film.


• Sjögren's patients with dry eye should carefully clean their eyelids with warm water or one of the commercially available eyelid cleansers.


• Use non-preserved artificial tears frequently and regularly, even when your eyes feel good. The goal is to keep your eyes comfortable, not to wait until they are uncomfortable.


• Keep the upper and lower eyelids free of facial creams at bedtime; they can enter the eye and cause irritation.


• Dry eye patients often develop or aggravate their environmental allergies. An over-the-counter allergy drop (even if preserved) used twice daily may help.


• Try ointments or gels at bedtime by first applying them only to the eyelids and lashes. If that is not helpful, place ~1/4 inch of ointment between the lower lid and eyeball.


• Eye ointments and gels can blur your vision and are usually reserved for overnight use.


• For dry eye, apply a warm, wet compress to the closed eyes using a washcloth. Apply at bedtime and upon awakening for 5 minutes, or more often if helpful.


• If your vision is blurred with artificial tear use, try a less thick (viscous) drop or ointment.


• Try moisture chamber glasses, wrap-around sunglasses, or other glasses, goggles or shields to prevent moisture evaporation and offer protection from air currents that irritate your dry eye.

Dry Skin Coping Tips

• Take short, warm baths or showers to help with dry skin. Hot water removes skin oils.


• Sjögren's patients with dry skin should pat dry after bathing and moisturize immediately while the skin is still damp. You can use petroleum jelly, bath oil or even some cooking oils such as safflower oil, olive oil, Canola® oil and Crisco®.


• Drag moisture into your skin by using products that contain chemilas such as urea, glycerin, lactic or similar "metabolic" or alpha-hydroxy acids, such as AmLactin® cream or Carmol®.


• Sjögren's patients with dry and/or sensitive skin should avoid fabric softeners in the washer and dryer.


• After swimming, make sure that you shower and then immediately use a moisturizer to reduce dry skin symptoms.

Sun & Sjögren's Survival Tips

• To reduce reactions to the sun, wear good UV-protective eye lenses and sunglasses, and seek the shade when outside.


• Protect your skin and eyes through use of sunscreen, sunglasses, ultraviolet light-protective clothing, hats, and non-fluorescent lighting.


• Did you know that Sjögren's patients can react to the sun and other sources of ultraviolet (UV) light? Consider purchasing UV-protective car and home window films that are clear or tinted to protect yourself from UV radiation.


• Look for the words "broad spectrum" on sunscreen protection. This means that you will be protected from both UVA and UVB radiation.


• Sjögren's patients who react to the sun should be especially careful to use sunscreen that protects against both UVA and UVB rays. Doctors now recognize the dangers of UVA light in addition to those of UVB.


• Remember that water, humidity and sweating decrease sunscreen effectiveness and mean you must reapply your sunscreen. Sjögren's symptoms can be aggravated by sunlight.


• Did you know that ultraviolet (UV) radiation from the sun and other light sources can affect Sjögren's patients, leading to skin rashes, disease flares, eye sensitivity and pain? Protect yourself using tips offered by the SSF.

Fatigue Coping Tips

• Battling fatigue with Sjögren's? Know your limits and pace yourself.


• Don't be a couch potato! A common cause of chronic pain and fatigue in any disorder, including Sjögren's syndrome, is lack of exercise.


• Educate your friends and family about what you are going through and how fatigue in Sjögren's syndrome can come and go.


• Work with your doctor to find a specific cause and treatment for your fatigue. Sjögren's can cause fatigue, but there can be other related causes.

Brain Fog Coping Tips

• Always report changes in cognition/memory and mood, such as depression and anxiety, to your doctor. "Brain fog" can accompany Sjögren's, but all changes in usual symptoms should be reported.


• Did you know that "brain fog" is a major complaint of Sjögren's patients? Hint: Train the brain! If you don't use it, you will lose it.


• Don't assume your "brain fog" is due to Sjögren's, especially in patients over 65-70 years of age: a major cause of cognitive dysfunction can be side effects of drugs and drug interactions.


• To help symptoms of "brain fog," minimize stress and anxiety. Take breaks throughout the day and learn relaxation exercises and practice them at regular intervals.


• Reduce caffeine and alcohol to help with "brain fog" and sleep problems in Sjögren's.


• Boost your brain power: Continue to work into retirement (part time) if able, learn new skills, volunteer, engage in social and mentally stimulating activities and establish new friendships and relationships.


• Letting yourself laugh and talk about your feelings will help reduce stress and anxiety, which contribute to fatigue and "brain fog" in Sjögren's.

Sleep & Sjögren's Coping Tips

• Sjögren's patients who have trouble sleeping should make sure the bedroom is comfortable, secure, dark and quiet.


• To maintain good "sleep hygiene," get out of bed at the same time every morning and into bed with lights out at the same time every night.


• Did you know that sleep problems are a major complaint among Sjögren's patients? To help with sleep, avoid alcohol and caffeine after 4:00 p.m.

Surgery & Sjögren's Coping Tips

• Sjögren's patients undergoing surgery should talk to their doctor about bringing medications and OTC products that are not commonly stocked in hospital pharmacies.


• If you are headed to the hospital, you should inform hospital personnel during the pre-admission visit or phone call about the OTC and prescription medications you need and are bringing.


• Consider using a nasal moisturizer before surgery, even if you do not routinely use one. Sjögren's patients tend to be drier than expected after surgery.


• Anticipate experiencing more dryness than usual after surgery. Sjögren's patients should bring extra eye drops and oral and nasal moisturizers to the hospital.


• Tell your surgeon, anesthesiologist and other physicians and staff involved in your hospital care that you have Sjögren's. Share information about your dryness and other symptoms and routine care.


• If you are headed for surgery, ask the nurse to speak with the anesthesiologist to obtain permission for you to take Evoxac® or Salagen® the morning of your surgery if it is part of your routine care.


• Before entering the hospital, check out your hospital's policies. You may be allowed to keep your medications at your bedside, or they may be administered by a nurse. OTC products probably can be kept at your bedside for use as needed.

Reflux & Sjögren's Coping Tips

• For nighttime reflux, avoid eating at least 3 hours before bed and limit liquids to small sips to keep the stomach empty.


• Elevate the head of your bed by raising the bed posts at least 30-45 degrees to help with nighttime reflux. Do not use extra pillows to elevate the head.

Dry Nose & Sjögren's Coping Tips

• Try nasal irrigation for a dry nose. Instructions for various methods are available through the SSF.


• Use a moisturizing spray or gel for your dry nose.

Dry Ear Coping Tips

• A drop of earwax remover or mineral oil can help dry itching ears.