Sjogren's Syndrome

What Is Sjogren's Syndrome?

Sjogren’s syndrome is an autoimmune disease that causes your immune system to go haywire and attack healthy cells instead of invading bacteria or viruses. Your white blood cells, which normally protect you from germs, attack the glands that are in charge of making moisture. When that happens, they can't produce tears and saliva, so your eyes, mouth, and other parts of your body dry out. There are treatments that bring relief, though.

It's natural to worry when you learn you've got a lifelong disease that will need regular care. Keep in mind that most people with Sjogren's stay healthy and don't have serious problems. You should be able to keep doing all the things you love to do without making many changes.

Sjogren’s Syndrome Causes and Risk Factors

Doctors don't know the exact cause. You may have genes that put you at risk. An infection with a bacteria or virus may be a trigger that sets the disease in motion.

For example, let's say you have a defective gene that's linked to Sjogren's, and then you get an infection. Your immune system swings into action.

White blood cells normally lead the attack against the germs. But because of your faulty gene, your white blood cells target healthy cells in the glands that make saliva and tears. There's no let-up in the fight, so your symptoms will keep going unless you get treatment.

Some other things can make you more likely to have Sjogren’s, including:

  • Age. Sjogren’s usually affects people over 40, but younger adults and children can get it, too.

  • Gender. Women are 10 times more likely to have Sjogren’s than men.

  • Other autoimmune issues. Nearly half of all people who have Sjogren’s also have another autoimmune condition, like lupus and rheumatoid arthritis.

Sjogren’s Syndrome Symptoms

The symptoms of Sjogren's can be different from person to person. You may have just one or two, or you may have many. By far, the most common symptoms are:

  • Dry mouth that may have a chalky feeling or feeling or feel like cotton 

  • Dry eyes that may burn, itch, or feel gritty

  • Dry throat, lips, or skin

  • Dryness in your nose

  • A change in taste or smell

  • Swollen glands in your neck and face 

  • Skin rashes and sensitivity to UV light

  • Dry cough or shortness of breath

  • Feeling tired

  • Trouble concentrating or remembering things

  • Headache

  • Dryness in the vagina in women

  • Swelling, pain, and stiffness in your joints 

  • H eartburn, a sensation of burning that moves from your stomach to your chest

  • Numbness or tingling in some parts of your body

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Sjogren’s Syndrome Diagnosis

Because so many people with Sjogren's also have another autoimmune disease, and Sjogren’s symptoms sometimes look a lot like some other diseases, like fibromyalgia or chronic fatigue syndrome, it can sometimes be hard for your doctor to give you a diagnosis.

To get clues, your doctor will give you a physical exam and may ask you questions such as:

  • Do your eyes itch or burn often?

  • Are you getting a lot of cavities in your teeth?

  • Does your mouth get dry? How about your lips?

  • Do you have stiff or painful joints?

Your doctor may ask you to get some blood tests. They will take some blood from your vein and send it to a lab to get checked.

The blood tests measure the levels of  the different types of blood cells you have and can show if you have germ-fighting proteins (antibodies) that many people with Sjogren's have. They can also measure inflammation in your body and the amount of certain proteins called immunoglobulins that are part of your body’s infection-fighting system. High levels of these can be signs that you have thedisease.

Your blood tests can also give your doctor an idea of how well your liver is working and show if there might be any issues with it. 

Your doctor also may recommend a few tests related to your eyes and mouth:

  • Schirmer tear test. This measures how dry your eyes are. Your doctor will put a small piece of paper under your lower eyelid to see how much your eye tears up.

  • Slit lamp. Your doctor uses this magnifying device to get a close look at the surface of your eye.

  • Dye tests. Your doctor puts drops of dye in your eyes to check for dry spots.

  • Salivary flow test. This measures the amount of saliva you make over a certain amount of time. 

  • Salivary gland biopsy. Your doctor will take a tiny piece of a salivary gland, usually from your lower lip, for testing. This can tell them if you have a rare condition called  lymphocytic infiltrate, which is a buildup of white blood cells that look like bumps.

In some cases, they also may suggest an imaging test:

  • Sialogram. Your doctor uses this to show how much saliva flows into your mouth. They’ll give you a shot of dye in the salivary glands in front of your ears and use a special kind of X-ray to take pictures of its flow.

  • Salivary scintigraphy. This imaging test is used to track how quickly a tiny amount of a radioactive substance gets to all of your salivary glands. Your doctor will give you a shot of the substance then track its progress over the next hour.

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Questions for Your Doctor About Sjogren’s Syndrome

  • What can I do for my dry eyes?

  • Is there anything I can do to moisten my mouth?

  • What can I do for joint pain?

  • Because of my immune system problem, is it safe for me to get a flu shot?

Sjogren’s Syndrome Treatment

You'll need to take medicine throughout your life to help you manage your symptoms. You can buy some kinds in a drugstore without a prescription, while your doctor may need to prescribe stronger ones if those don’t work well enough.

For instance, drops called " artificial tears" can keep your eyes from drying out. You'll need to use them regularly throughout the day. There are also gels that you put on your eyes at night. The advantage of the gels is that they stick to your eye's surface, so you won't need to apply them as often as the drops.

If artificial tears aren't helping, your doctor may prescribe drugs for your dry eyes, including:

  • Cequa

  • Lacrisert

  • Restasis

Lacrisert is a tiny rod-shaped medicine. You put it into your eye with a special applicator, usually once or twice a day. Cequa and Restasis come in drops, which you use twice a day.

Another treatment option for dry eyes is a procedure called punctal occlusion. This is when your doctor puts tiny plugs into your tear ducts to block them up. This keeps tears from draining away too fast, meaning they stay on your eyes longer and help your eyes stay moist. 

To help your dry mouth, your doctor may prescribe drugs that boost the amount of your saliva, including:

There are other treatments for some of the less common symptoms of Sjogren's syndrome. For instance, if you get yeast infections in your mouth, your doctor might prescribe antifungal medicine.

If you get heartburn, your doctor may give you medicines that curb the amount of acid in your stomach.

Your doctor may also suggest a medicine called hydroxychloroquine (Plaquenil) to treat your joint pain. It's a drug that's also used to treat malaria, lupus, and rheumatoid arthritis.

It's rare, but some people with Sjogren's get symptoms throughout the body, including belly pain, fever, rashes, or lung and kidney problems. For those situations, doctors sometimes prescribe prednisone (a steroid) or an anti-inflammation drug called methotrexate (Rheumatrex, Trexall).

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Sjogren’s Syndrome Home Remedies

There are lots of steps you can take on your own to help manage your symptoms.

For dry mouth:

  • Sip water frequently. 

  • Chew gum or suck on candy to stimulate saliva flow and help keep your mouth moist. Be sure they're sugar-free so you don't get cavities.

  • Try artificial saliva gels or sprays, especially before bed.

  • Ask your doctor, dentist, or pharmacist about mouthwashes or sprays that can relieve dryness. You might need to try several until you find a product that works for you.

  • Keep up with tooth brushing and flossing to avoid getting cavities. 

  • Schedule regular checkups with your dentist.

For dry eyes, nose, or skin:

  • Use a humidifier or vaporizer at night. These machines also can help your dry mouth or nose. 

  • Try a nasal saline spray or gel for dry nose.

  • Use warm water, not hot, when you bathe or shower if you have dry skin. Instead of using a towel after showering, let yourself "drip dry." Your skin will absorb the moisture from the shower.

Sjogren’s Syndrome Complications

Since you don't have enough saliva, which helps protect your teeth from decay, there's a chance you may get more cavities than other people. You could also get inflammation of your gums, called gingivitis, or yeast infections in your mouth. You also may find it hard to swallow. Dryness in your nose can lead to issues like nosebleeds or sinusitis.

 Dry eyes can make you more likely to get infections around your eyes that can harm your cornea. You also may notice some new issues with your vision.

Other less common health conditions linked to Sjogren’s include:

  • Irritable bowel syndrome (IBS)

  • Interstitial cystitis

  • Problems with your liver, like chronic active autoimmune hepatitis or primary biliary cholangitis

  • Conditions that affect your lungs, like bronchitis or pneumonia

  • Inflammation in your lungs, liver, and kidneys

  • Lymphoma, or cancer of the lymph nodes

  • Nerve issues

Sjogren’s Syndrome Outlook

You'll need to keep up with medicine throughout your life to get relief from symptoms.

Everyone's experience is different. With the right care, you can lead an active life.

It can help to get in touch with others who are going through the same thing. You can compare notes about symptoms and get ideas about what brings relief. Talk to your doctor about support groups in your area. Reach out to family and friends, too. They can be a great source of emotional support.

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Getting Support for Sjogren’s Syndrome

The Sjogren's Syndrome Foundation can connect you with others through support groups and conferences.

WebMD Medical Reference Reviewed by Hansa D. Bhargava, MD on June 18, 2020

Sources

SOURCES:

Sjogren's Syndrome Foundation: "About Sjogren's Syndrome."

American College of Rheumatology: "Sjogren's Syndrome," "Methotrexate."

National Institute of Arthritis and Musculoskeletal and Skin Diseases: "Questions and Answers about Sjogren's Syndrome."

NHS: "Treating Sjogren's Syndrome.”

Johns Hopkins Medicine: “Who’s At Highest Risk of Developing Sjogren’s Syndrome?”

Sjogren’s Foundation: “Understanding Sjogren’s: Symptoms,” “Diagnosis,”

Mayo Clinic:“Sjogren’s Syndrome.”

National Organization for Rare Disorders: “Lymphocytic Infiltrate of Jessner.” 

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