Sjogren's Syndrome

What Is Sjögren's Syndrome? Symptoms, Causes, Diagnosis, Treatment, and Prevention

Katherine Lee
By Medically Reviewed by 
Reviewed:
Medically Reviewed

Sjögren's (pronounced SHO'-grenz) syndrome is a chronic autoimmune disease in which the body’s immune system attacks moisture-producing glands, such as those that produce saliva and tears. This is why dry eyes and mouth are two of the most common symptoms of Sjögren's syndrome. The disease also affects other areas of the body, including the kidneys, blood vessels, joints, and the brain.

In about half of all cases, Sjögren's syndrome occurs by itself, and is called "primary Sjogren's syndrome." In the other half, the disease occurs alongside another autoimmune connective tissue disease, such as rheumatoid arthritis, lupus, or scleroderma, in which case it is called "secondary Sjögren's syndrome," according to the American College of Rheumatology.

Sjögren's syndrome is considered one of the more common autoimmune disorders. It's been estimated that there are approximately four million people in the United States with Sjögren's syndrome, notes the Sjögren’s Foundation.

Tennis champion Venus Williams, who made her diagnosis public in 2011, is one of them.

Signs and Symptoms of Sjögren's Syndrome

Symptoms of Sjögren's syndrome vary from one individual to another, but according to the Arthritis Foundation, common symptoms include:

Eye Symptoms

  • Dry eyes
  • Burning or stinging in the eyes
  • Blurry vision
  • Sensation that foreign particles or something gritty is in the eyes
  • Sensitivity to light
  • Blephartis (inflammation in the eyelids)

Mouth, Throat, and Nose Symptoms

  • Dry mouth (which may include thick and stringy saliva)
  • Swollen salivary glands
  • Mouth sores
  • Dental issues, such as cavities and gum inflammation
  • Altered sense of taste and smell
  • Dry nasal passages or sinusitis
  • Dry or burning throat
  • Difficulty swallowing
  • Hoarseness, difficulty speaking
  • Dry cough

Other Symptoms

Causes and Risk Factors of Sjögren's Syndrome

Researchers aren't sure exactly what causes Sjögren's syndrome, but they believe that a combination of genetic and environmental factors may predispose someone to developing this condition, according to the National Institute of Dental and Craniofacial Research (NIDCR). Several genes appear to be involved in the development of Sjögren's, and a previous bout of a viral or bacterial infection may play a role in triggering the disease.

In people who are genetically predisposed, an infection may act as a trigger to activate their immune system and cause it to attack healthy cells and tissue, notes the Mayo Clinic.

There is also some evidence that having a relative who has Sjögren's syndrome may increase your risk: According to the Arthritis Foundation, approximately 12 percent of people with Sjögren's have one or more relatives who also have the disease.

And since Sjögren's affects women at a far greater rate than men — 9 out of 10 people with the disease are women, according to the Sjögren's Foundation — it's believed that estrogen may play a role in the development of the disease.

Being a post-menopausal woman is a risk factor for Sjorgen's syndrome.

How Is Sjögren's Syndrome Diagnosed?

Diagnosing Sjögren's syndrome can be particularly challenging because many of the symptoms of this complex disease mimic those of a wide variety of other conditions, including fibromyalgia and chronic fatigue syndrome, according to Johns Hopkins Medicine.

Since there are many different types of symptoms, and someone may experience some symptoms but not others, it may be difficult for a healthcare provider to see the entire picture. For instance, you may see an ophthalmologist for dry eye symptoms and a dentist for mouth issues, and then another doctor for joint pain, but each individual doctor may not register all of your symptoms and recognize that they're due to a single unifying diagnosis of Sjögren's syndrome.
There's no single test to diagnose Sjögren's, but specific antibodies need to be present in your blood and a pattern of inflammation needs to be observed.

To diagnose Sjögren's syndrome, a rheumatologist — the specialist who typically treats this condition — will usually start by taking your medical history and conducting a physical exam. Depending on those results, your doctor may order tests, such as:
  • Blood Tests These look for specific antibodies common in Sjögren's syndrome as well as evidence of other autoimmune disorders, such as lupus and rheumatoid arthritis.
  • Biopsy Your doctor may remove tissue or cells from your salivary glands in your lip to send to a lab to check for signs of inflammation.
  • Imaging Tests These may include an ultrasound or magnetic resonance imagine (MRI), on the salivary gland.
  • Sialometry This noninvasive test measures the amount of saliva you can produce over a set period of time.
  • Eye Tests These tests will typically be performed by an ophthalmologist and may include a Schirmer's test, which measures the amount of tears your eyes make over a certain period of time, and a dye test that can help your doctor see how dryness has affected your cornea and other parts of your eye.

Prognosis of Sjögren's Syndrome

Your prognosis depends on your symptoms and the severity of your disease. Some people may only experience dry eyes and mouth, while others may have more serious symptoms that can go through periods of remissions and flare-ups, according to the National Institute of Neurological Disorders and Stroke (NINDS). While symptoms like dry eyes and mouth can be uncomfortable and inconvenient, they can often be managed well with appropriate treatments.

How soon your condition is identified and treated can also play a role in how much you are impacted by the disease. Early diagnosis is important for reducing the effects of Sjögren’s disease. Most patients with Sjögren's remain healthy and the defining symptoms of dry eyes and mouth can also get better with time, according to the American College of Rheumatology (ACR).

For people whose Sjögren's syndrome is moderate to severe, symptoms such as eye discomfort, recurrent mouth infections, difficulty swallowing, joint pain, and debilitating fatigue can all have a negative impact on quality of life.

When Sjögren's syndrome has been very active for a long period of time, there is an increased risk for developing lymphoma and early death, according to the U.S. National Library of Medicine.

And according to the National Institute of Health's Genetic and Rare Diseases Information Center (GARD), the risk of death is also increased when someone has conditions associated with Sjögren's syndrome, such as systemic lupus erythematous (SLE) or rheumatoid arthritis.

Duration of Sjögren's Syndrome

Sjögren's syndrome is a lifelong illness. There is no cure, but most people who have the condition can work with their rheumatologist to find the best treatment to manage their symptoms.

Treatment and Medication Options for Sjögren's Syndrome

Your doctor may suggest the following strategies and treatments:

Practice good oral hygiene. Dryness in the mouth can increase your risk for cavities and infections, which is why dental problems are common in people with Sjögren's syndrome. To prevent tooth decay and other problems, brush your teeth after every meal and before bedtime, and floss every day. Your doctor may also recommend prescription toothpastes or mouthwashes that are formulated to treat dry mouth, according to Johns Hopkins Medicine.

Prevent dry mouth. Take sips of water frequently throughout the day and chew sugar-free gum or suck on sugar-free candy to keep your mouth moist and boost saliva production. (Avoid sugary foods and drinks as they can lead to tooth decay.)

Try to limit or avoid coffee, alcohol, and nicotine, which can irritate and dry out your mouth, and use a lip balm to prevent parched or cracked lips.

Keep your eyes moist and protected. Your doctor may recommend using lubricating eye drops or ointments to help keep your eyes moist. According to the American Academy of Ophthalmology, it's important to treat dry eyes if you have Sjögren's syndrome because ongoing irritation can, in rare cases, lead to infection and scarring and may affect your vision.

If your eye dryness is severe and persists, talk to your doctor about other treatments. One option may be punctal occlusion, in which your tear ducts are sealed with collagen or silicone plugs to block the drainage of tears in order to increase lubrication in your eyes, according to the Mayo Clinic.

Related: 8 Do-It-Yourself Ways to Manage Dry Eye

Find an exercise plan that protects your joints. If you have joint pain or swelling, talk to your doctor or physical therapist to find exercises that can help you relieve joint stiffness and improve range of motion and flexibility, suggests the Arthritis Foundation.

Take a look at your diet. Many people with Sjögren's syndrome may experience problems related to eating. Some people may have trouble eating certain foods due to dryness and swelling of the throat, while others may have digestive issues, such as heartburn and acid reflux, which are common in people with Sjögren's, notes the Cleveland Clinic.

If you're experiencing gastrointestinal symptoms, talk to your rheumatologist and a registered dietitian to identify which foods you should avoid and what you should include in your diet to make sure you get the nutrients you need. Sometimes this requires keeping a so-called food diary, in which you record what you eat and try to correlate that with your symptoms.

Medication Options

Medication may be required for people who develop symptoms such as joint pain, fatigue, fibromyalgia, or more serious symptoms related to inflammation involving organs such as the kidneys, intestines, lungs, or the brain. According to NYU Langone Health, medications that may be recommended include:

  • Mild Pain Relievers for Muscle and Joint Pain These include nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen and naproxen.
  • Cholinergic Agonists Your doctor may prescribe these medications, which help to stimulate secretions in the body, to help create more tears and saliva.
  • Vaginal Dryness Remedies Doctors may recommend over-the-counter vaginal moisturizers or prescription estrogen (in cream, ring, or tablet form) for those who may be experiencing vaginal dryness.
  • Steroids, Such as Predisone, to Treat Flares of Symptoms Like Joint Pain or Rashes While prednisone and other steroids are effective at decreasing inflammation, they have side effects — such as weight gain and high blood pressure and osteoporosis — associated with higher doses and long-term use.
  • Immunosuppressant Drugs for Joint Pain. As their name suggests, these medications suppress an overactive immune system. Your doctor may initially prescribe hydroxychloroquine; methotrexate may be considered next. In more severe cases of Sjögren's, in which organs are affected, doctors may prescribe more powerful immunosuppressants, such as cyclophosphamide and rituximab.

Alternative and Complementary Therapies

  • Acupuncture for Dry Eye A review of studies published in May 2018 in BMC Complementary Medicine and Therapies found that acupuncture was more effective than artificial tears in treating dry eye syndrome.

  • Paraffin Bath Applying moist heat to your hands and wrists in the morning by immersing them in warm wax may help with stiffness, according to the Johns Hopkins Sjögren's Center.

  • Gentle Exercises The Johns Hopkins Sjögren's Center also notes the benefits of tai chi, yoga, and dancing to strengthen muscles and help maintain range of motion.

  • Nutritional Supplements Some research, such as study published in November 2016 in the journal Cornea, has suggested that omega-3 supplements may help improve symptoms associated with dry eye.

Prevention of Sjogren's Syndrome

There is no known way to prevent Sjögren's syndrome, but there are treatments and measures you can take to alleviate your symptoms such as dry eyes and mouth and joint pain.

Complications of Sjögren's Syndrome

Sjögren's syndrome affects the entire body, causing symptoms that go beyond dryness in the mouth and eyes. This disease can affect the gastrointestinal system, the central nervous system, and organs such as the kidneys, liver, pancreas, lungs, and more. Some common complications include:

  • Damage to the eye
  • Dental cavities, oral infections
  • Pulmonary disease
  • Neuropathy
  • Interstitial cystitis (painful bladder syndrome)
  • Lymphoma
  • Kidney problems
  • Vasculitis (rare)
  • Problems in pregnancy (women with Sjögren's syndrome have a higher risk for complications during pregnancy), according to a 2017 review in The Permanente Journal

Research and Statistics: Who Has Sjögren's Syndrome?

Sjögren's syndrome can affect people of any age, including children, but the disease is usually diagnosed in middle age. Most people are diagnosed between age 40 and 69.

And while it can affect men, the disease is much more prevalent in women. As mentioned above, as many as 9 out of 10 people who have Sjögren's are women.
The Sjögren's Foundation notes that an estimated four million Americans have the disease.

Related Conditions of Sjögren's Syndrome

According to StatPearls, some of the conditions commonly associated with (and mistaken for) Sjögren's include:

Resources We Love

Sjögren's Foundation

This national nonprofit foundation focuses on increasing education, awareness, and research about this condition and providing support and information to people with Sjogren's and their loved ones.

National Institute of Dental and Craniofacial Research

Where can you go for info on the latest research on Sjögren's syndrome? How can you find a clinical trial? This National Institutes of Health site is an excellent hub of information and reliable resources for Sjögren's syndrome.

UNC School of Medicine's Autoimmune Disease Podcast

This 12-part series on autoimmune diseases includes an episode on Sjogren's Syndrome. Beth Jonas, MD, an associate professor of medicine in the division of rheumatology, allergy, and immunology at UNC School of Medicine, discusses the disease and how to manage it.

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