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Sjögren's Disease (Syndrome)

Sjögren’s disease is an autoimmune condition where your immune system, which normally fights infections, mistakenly attacks your own body. Sjögren’s mainly affects the glands that produce moisture, like your tear and saliva glands, leading to dry eyes and a dry mouth. However, Sjögren’s can also cause joint pain, fatigue, and sometimes affects other parts of the body, including organs like the lungs and kidneys.

Early diagnosis and treatment can prevent serious impairment and improve your quality of life.


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What Is Sjögren’s Disease?  

Sjögren’s (pronounced SHOW-grins) disease, previously referred to as Sjögren’s syndrome, is a systemic autoimmune disorder that causes damage to the moisture-producing glands. The disease is nearly as common as rheumatoid arthritis.

In Sjögren’s disease, the immune system mistakenly attacks the body’s own healthy tissues, damaging the glands that make tears and saliva. The result is chronic dryness throughout the body but most commonly in the eyes and mouth.

While Sjögren’s typically affects the secretory glands, it’s a systemic disease that can affect nearly any organ.

Because it affects the entire body and symptoms are often not specific, Sjögren’s disease can be hard to diagnose. People often receive a delayed diagnosis because Sjögren’s symptoms mimic many other conditions.

Some of the conditions Sjögren’s disease ismost commonly mistaken for are:

Further delaying diagnosis are different approaches to treatment from different doctors. For example, your eye doctor may treat your dry eye but not recognize your skin symptoms. Likewise, your primary care provider may treat your joint pain but not know you have dry eye.  

Sjögren's can occur on its own without another autoimmune disorder or with another autoimmune condition. Half of cases occur with another autoimmune condition, such as lupus, rheumatoid arthritis, or scleroderma. While some people experience only mild effects, others have disabling symptoms.

What causes Sjögren’s disease?  

The cause of Sjögren’s disease is unknown but likely involves a combination of genetic and environmental risk factors, such as viral or bacterial infections.

What are Sjögren’s disease risk factors?

According to the National Institutes of Health (NIH), the risk factors for Sjögren’s include:

  • Being age 50 or older.
  • Being assigned female at birth.
  • Having a family history of autoimmune disease in first-degree relatives.
  • Having a mutation in certain genes.
  • Having a personal history of other autoimmune diseases.
  • Other environmental factors – Certain infections, especially viral infections, may trigger the immune system in a way that leads to Sjögren’s in genetically susceptible individuals. However, this link isn’t fully understood.

Complications of Sjögren’s disease  

If left untreated, Sjögren’s disease can cause discomfort and sometimes additional health issues.

These include:

  • Cancer, particularly non-Hodgkin lymphoma. People with primary Sjögren’s disease have a 15 to 20 times higher risk of developing non-Hodgkin B-cell lymphoma compared to the general population.
  • Dental decay and tooth loss.
  • Inflammatory arthritis.
  • Kidney disease.
  • Lung problems.
  • Neurologic involvement.
  • Permanent eye problems.
  • Pregnancy complications, although rare, such as a rash on the baby and heart block.

How common is Sjögren’s disease? 

Sjögren's disease is often considered a rare disease, but millions of people are estimated to be living with the disease – making it one of the more prevalent autoimmune diseases.

The disease affects people of all races and ethnicities. Most are ages 50 or older when they're first diagnosed, and women are nine times more likely to have Sjögren's disease than men.

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What Are the Signs and Symptoms of Sjögren’s Disease?

Just as every person is different, every person's Sjögren’s manifestations also are different. Some people have constant symptoms, while others have symptoms that come and go. Still, others have cycles of mild and serious symptoms.  

Two symptoms that people most share are:

  • Dry eyes — Your eyes may burn, itch, or feel gritty. Your vision may be blurry, or your eyes may be sensitive to bright lights.
  • Dry mouth — You may have trouble talking, swallowing, and tasting. You may have a dry tongue and throat.

Sjögren’s disease also can cause symptoms in other body parts, such as:

  • Brain fog.
  • Dry, lingering cough.
  • Dry skin.
  • Fatigue.
  • Joint pain.
  • Muscle pain.
  • Numbness in feet or hands.
  • Rashes.
  • Swelling of the glands.
  • Vaginal dryness.

When should I see a doctor about my Sjögren’s Disease symptoms? 

If you're experiencing symptoms of Sjögren’s Disease that are causing discomfort or affecting your activities of daily living, it’s time to talk to your doctor. If you've been diagnosed with Sjögren’s disease, you should be monitored by a rheumatologist — a doctor specializing in autoimmune disorders.

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How Do You Diagnose Sjögren’s Disease?  

A rheumatologist can diagnose Sjögren’s disease. But it’s not uncommon for your ophthalmologist (eye doctor), dentist, or other specialists to perform tests that help in the diagnosis.

A Sjögren's diagnosis is based on how well your salivary and tear glands work and evidence of autoimmunity (immune markers associated with Sjögren’s and inflammation within the saliva-producing glands). Your provider may run lab tests, imaging scans, or perform a biopsy to confirm Sjögren’s or another autoimmune disorder.

What to expect during your visit

No single test can confirm a Sjögren’s diagnosis, so your doctor will ask you about your symptoms.

They'll ask you about the symptoms that are associated with Sjögren’s extraglandular manifestations and about rheumatologic conditions more broadly.

For instance, they may ask you:

  • If your eyes are dry.
  • If your mouth is dry.
  • When the dryness started.
  • Whether you feel it all the time or occasionally.

They may also run tests, including:

  • Blood tests — To detect the presence of Sjögren’s disease-associated antibodies and evidence of immune activation. For example, more than 70% of people with Sjögren’s are positive for the SS-A (or Ro) marker.
  • Eye tests — Lissamine green and fluorescein dye tests and the tear break-up time measurement check for dry spots on the eye’s surface. The Schirmer test used to measure tear production and tear osmolarity also can be used to test for dry eye.
  • Imaging tests — To diagnose Sjögren’s. Salivary gland ultrasounds assess salivary gland structure. Less commonly used is parotid scintigraphy, which evaluates gland function and sialography; x-ray/CT scan using dye inserted into the glands; or noninvasive MRI to outline the salivary duct anatomy and detect dye that’s been inserted into the glands. If you have pulmonary symptoms, a high-resolution CT scan also is recommended.
  • Oral tests — Salivary flow tests to measure saliva production and salivary gland biopsy taken from the lower lip to confirm lymphocytic infiltration of the minor salivary glands.
  • Pulmonary function tests — To measure how well your lungs work. They are recommended for patients with pulmonary symptoms or findings on a different exam.

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How Do You Treat Sjögren’s Disease?  

Each person’s symptoms and manifestations of Sjögren’s aren't the same, so Sjögren’s treatment plans are different, too. The goal is to reduce side effects and improve your health in the short and long term.

Your treatment will depend on which parts of your body are affected by Sjögren’s.

Treatment plans often include a combination of self-management approaches and over the counter (OTC) and prescription medications. Sometimes, lifestyle changes can relieve certain symptoms, such as fatigue.

Medicines to treat Sjögren’s disease

Prescription medicine may be needed at some point to control your disease progression and reduce complication risks.

No single medication has proved to slow the disease progression of Sjögren’s or treat all symptoms. Because there's no cure, treatment concentrates on relieving symptoms and preventing complications from the overactive immune system. Make sure to tell your doctor which medications you currently take because some may worsen eye and mouth dryness.

Your provider will work with you, your dentist, your ophthalmologist, and other subspeciality providers to develop a personalized plan to treat your specific symptoms.

For eyes

  • Artificial tears (eyedrops) — Moisten and lubricate dry eyes. You may have to try a few to find the best eye drops for you.
  • Ointments Thicker than eye drops, ointments can moisten the eyes for several hours. Because they can give you blurry vision, they’re best used right before bedtime.
  • Prescription eyedrops — Contain medication to reduce swelling in the eye and suppress the immune system.
  • Tear duct plugs — Placed in the tear ducts in the corners of the eyes, duct plugs block drainage to keep tears in the eyes longer. A simple procedure that can be done in the ophthalmologist’s office, having duct plugs placed takes only a few minutes and can bring dry eye relief.
  • Scleral lenses — These arch over the cornea to create a space that’s then filled with saline solution. The lenses act as a protective shield to hold in moisture.

Mouth

  • Saliva production stimulators — Medications that ramp up the salivary glands to produce more saliva and tears.
  • Artificial saliva — Available by prescription, a saliva substitute can help keep the mouth feeling moist.
  • Antifungals — Treat fungal infections, such as candidiasis (thrush), which often affect people with dry mouths.
  • Prescription-strength fluoride toothpaste — To prevent cavities.

System features

  • Disease-modifying antirheumatic drugs (DMARDs) and antimalarial drugs — These haven't been approved specifically for treating Sjögren’s but may help relieve joint pain and rash in some people with extraglandular features of the disease.
  • Biologics or small molecule drugs — Certain biologics, like Rituximab, and drugs that inhibit B cell-activating factor (BAFF), such as Belimumab, target B cells, which play a role in Sjögren’s disease.
  • Corticosteroids — Help control inflammation and pain but are not for long-term use. They are usually prescribed at the lowest possible dose to achieve relief from the most serious symptoms.
  • OTC or prescribed pain relievers — Nonsteroidal anti-inflammatory drugs (NSAIDs) can ease swollen gland discomfort and alleviate joint and muscle pain.

Why Choose UPMC for Sjögren’s Disease Care?  

UPMC is home to the first dedicated Sjögren’s disease center in the greater Pittsburgh region.

Here, you can expect seamless care across multiple disciplines. We partner with the UPMC Vision Institute on a monthly dry eye clinic at UPMC Mercy Pavilion.

Our experts are also building a research program where they can learn and better understand Sjögren’s diseaseand conduct research that will fuel the development of future therapies.

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Last reviewed by Rachael Gordon, MD, PhD on 2024-12-02.