Sjögren's Syndrome

What is Sjögren’s syndrome?

Sjögren’s syndrome is an autoimmune disease that makes your glands produce less moisture than they should. It causes chronic (long-term) dryness throughout your body (especially your eyes and mouth). Sjögren’s syndrome is pronounced “SHOW-gren’s syndrome.”

If you have Sjögren’s syndrome, your immune system damages glands in your body that produce and control moisture, including in your:

• Tear system.
• Mouth (your salivary glands).
• Nose.
• Throat.
• Digestive system.
• Vagina.

Visit a healthcare provider if you’re experiencing new dryness in your eyes, mouth or anywhere else in your body — especially if you’re also experiencing other symptoms like pain.

Types of Sjögren’s syndrome

Healthcare providers classify Sjögren’s syndrome into two types:

• Primary Sjögren’s syndrome develops on its own, and isn’t caused by another health condition.
• Secondary Sjögren’s syndrome happens when another condition or issue causes (triggers) Sjögren’s syndrome.

How common is Sjögren’s syndrome?

Experts estimate that around 2 million people in the U.S. have Sjögren’s syndrome.

What are Sjögren’s syndrome symptoms?

The most common symptom of Sjögren’s syndrome is unusual dryness, including:

• Dry eyes or itchy eyes.
• Dry mouth (xerostomia), mouth sores or thickened spit (saliva).
• Vaginal dryness.
• Dry skin.
• Dry nose and frequent nosebleeds.
• Dry throat (including coughing frequently).

In addition to dryness, Sjögren’s syndrome can cause other symptoms, including:

• Joint pain.
• Muscle pain or weakness.
• Swollen lymph nodes.
• Fatigue (feeling tired all the time).
• Trouble swallowing (dysphagia) or talking.
• Brain fog (trouble thinking or focusing).
• Loss of taste.
• Tooth decay (cavities) or loss.
• Skin rashes.
• Neuropathy.
• Heartburn or other types of indigestion.
• Light sensitivity.

What causes Sjögren’s syndrome?

Sjögren’s syndrome is an autoimmune disease. Autoimmune diseases happen when your immune system mistakenly damages your body instead of protecting it. Experts aren’t sure what makes your immune system attack your glands and cause Sjögren’s syndrome.

Primary Sjögren’s syndrome happens with no known trigger or cause.

Other health conditions trigger secondary Sjögren’s syndrome, especially other autoimmune diseases and some viral infections.

Viral infections that can trigger secondary Sjögren’s syndrome include:

• Hepatitis C.
• Cytomegalovirus (CMV).
• Epstein-Barr virus.
• Human T-lymphotropic virus 1 (HTLV).
• COVID-19.

Any autoimmune disease can trigger secondary Sjögren’s syndrome. Some autoimmune diseases that are related to Sjögren’s syndrome include:

• Rheumatoid arthritis.
• Psoriatic arthritis.
• Lupus

Even though studies have linked Sjögren’s syndrome to other conditions, there’s no guarantee you’ll develop it if you have these conditions. Similarly, Sjögren’s syndrome might make you more likely to develop other autoimmune conditions, but that doesn’t mean you definitely will.

What are the risk factors for Sjögren’s syndrome?

Anyone can develop Sjögren’s syndrome, but certain groups of people are more likely to have it:

• Women and people assigned female at birth (AFAB). More than 90% of people with Sjögren’s syndrome are AFAB. People assigned male at birth (AMAB) can develop it, but it’s much less common.
• People with other autoimmune diseases. Around half of people with Sjögren’s syndrome have at least one other autoimmune condition.
• People between the ages of 45 and 55. Children, younger adults and adults older than 55 can have Sjögren’s syndrome, but it usually develops in adults in that age range.
• People who have a biological relative with Sjögren’s syndrome. Around 10% of people with Sjögren’s syndrome have a direct relative (a biological parent or sibling) with it, too.

How is Sjögren’s syndrome diagnosed?

A healthcare provider will diagnose Sjögren’s syndrome with a physical exam and some tests. They’ll examine your body and ask about your symptoms. Tell your provider when you first noticed changes in your body and if certain times of day or activities seem to make the symptoms worse.

Diagnosing Sjögren’s syndrome is usually part of a differential diagnosis. This means your provider will probably use a few tests to rule out other conditions and causes of your symptoms before diagnosing you with Sjögren’s syndrome. Some tests you might need include:

• Blood tests.
• An eye exam.
• A dental cleaning and checkup.
• Urinalysis.
• Biopsies.
• X-rays.

You might need to see a few specialists, including:

• A rheumatologist.
• Eye care specialists.
• A dentist.
• An otolaryngologist (ear, nose and throat specialist).

What are Sjögren’s syndrome complications?

Sjögren’s syndrome can cause some complications, including:

• An increased risk of oral and eye infections.
• Abnormal liver or kidney function.
• Lymphomas (cancerous tumors in the lymph nodes).
• Lung problems that may be mistaken for pneumonia.
• Neurological (brain, spine or nerve) problems that cause weakness or numbness.

Most people with Sjögren’s syndrome live their lives without experiencing severe complications. If you have secondary Sjögren’s syndrome, the condition that’s causing it might give you an increased risk of complications. Ask your provider what to expect.

How is Sjögren’s syndrome treated?

Your provider will suggest treatments to manage your symptoms. There’s no cure for Sjögren’s syndrome, but your provider will help you find a treatment plan that reduces its impact on your daily routine and quality of life.

Which treatments you’ll need depends on where Sjögren’s syndrome affects you.

Some common treatments for dryness in your eyes, mouth or vagina include:

• Artificial tears: Your eye care specialist might suggest over-the-counter (OTC) eye drops, prescription drops or lubricants to keep your eyes moist.
• Dry eye surgery (punctal plugs): An ophthalmologist (eye surgeon) can surgically close some or all of your tear ducts to keep your natural tears in your eyes for longer.
• Saliva producers: Your dentist may suggest over-the-counter saliva (spit) supplements or prescriptions to help your body make more saliva. They might suggest you chew gum, sip water throughout the day or suck on ice chips to help moisturize your mouth.
• Special mouthwash or dental care products: People with Sjögren’s syndrome have a higher risk of dental health issues, so you may need more frequent cleanings, special fluoride treatments or prescription toothpaste and mouthwash.
• Vaginal moisturizers or lubricants: Your provider will suggest over-the-counter or prescription lubricants to add moisture to your vagina every day or during sexual intercourse.
• Hormone therapy: Some people need hormone therapy (like supplemental estrogen) to treat vaginal dryness caused by a hormonal imbalance.

Treatments for pain and other symptoms can include:

• Over-the-counter pain relievers: Over-the-counter NSAIDs (nonsteroidal anti-inflammatory drugs) or acetaminophen can relieve pain and reduce swelling. Don’t take these medications for more than 10 days in a row without talking to your provider.
• Corticosteroids: Prescription anti-inflammatory medications your provider will give you as pills or injections.
• Immunosuppressants: Prescription medications that calm your immune system and reduce how active it is. Your provider might recommend disease-modifying antirheumatic drugs (DMARDs).

Can I prevent Sjögren’s syndrome?

Because experts don’t know what causes it, there’s no way to prevent Sjögren’s syndrome. There’s no way to know if (or when) someone will develop it.

What can I expect if I have Sjögren’s syndrome?

You should expect to manage your symptoms for a long time (maybe for the rest of your life). However, your symptoms should get less severe as you find treatments that work for you. People with Sjögren’s syndrome usually find that their symptoms become less intense over time because they learn how to manage them.

When should I see my healthcare provider?

See your provider if you notice new symptoms, or if your symptoms are getting more severe. Monitor your symptoms and keep track of any changes you notice.

Your provider will tell you how often you’ll need regular follow-up appointments, tests or additional screenings. Don’t hesitate to visit your provider if you think a treatment isn’t working (or isn’t as effective as it used to be). Sjögren’s syndrome symptoms can be subtle, and you’re the best judge of when something doesn’t feel quite right in your body.

Go to the emergency room or call 911 (or your local emergency services number) if you feel like you can’t breathe or swallow.

What questions should I ask my doctor?

Questions to ask your provider include:

• Which type of Sjögren’s syndrome do I have?
• Which tests will I need?
• Will I need to visit any specialists?
• Which treatments will I need?
• What changes or symptoms should I watch out for?

A note from Cleveland Clinic

Sjögren’s syndrome can be extremely annoying to manage. It’s frustrating to have dry eyes, a dry mouth or worry if having sex will hurt because of vaginal dryness. The good news is that most people with Sjögren’s syndrome are able to manage their symptoms very well once they find a combination of treatments that works for them.

Talk to your provider about adjusting your treatments if you feel like your symptoms are changing, getting worse or affecting your daily routine more than they should. Ask your provider how often you’ll need eye exams, dental cleaning or other exams and tests. These routine checkups can help you avoid complications.