Sjögren’s Disease: Diagnosing This Autoimmune Condition

Sjögren’s disease is a rare autoimmune disorder that causes inflammation in various parts of the body. It affects about 0.5% to 1% of people, and it’s 9 times more common in women than men.  

In Sjögren’s, the immune system causes inflammation in the glands that make tears and saliva. This inflammation results in dry eyes and dry mouth. In more severe cases, Sjögren’s can also cause inflammation in major organs like the brain, lungs, and/or kidneys. 

The name of primary Sjögren’s syndrome was recently changed to Sjögren’s disease (SJD). But many people are not yet aware of this change, which was intended to validate Sjögren’s as a disease. So it’s common to see both “disease” and “syndrome” as you read about it.

How do you diagnose Sjögren’s syndrome?

Rheumatologists — doctors who specialize in autoimmune disorders — commonly make the diagnosis of Sjögren’s disease. To arrive at a diagnosis, they collect clues from symptoms, a physical exam, and various tests.

What are the major symptoms of Sjögren’s?

To be diagnosed with SJD, you must have dry eyes and/or dry mouth. Your provider will ask specific questions about these symptoms. For example:

• Have you had problems with dry eyes every day for more than 3 months? 

• Do you feel like you always have sand or gravel in your eyes? 

• Do you use eye drops more than three times a day? 

• Have you had problems with dry mouth every day for more than 3 months?

• Do you need help from a glass of water in order to swallow a salty, dry cracker?

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If the answer is “no” to all of these questions, Sjögren’s disease is unlikely. If the answer is “yes” to some or all of these questions, you’ll probably need additional testing to get a diagnosis (more on this below).

Your provider will also check for other symptoms of Sjögren’s disease, like:

• Fatigue

• Muscle aches

• Joint pain or swelling

• Swollen salivary glands 

• Recurrent salivary gland infections or stones (sialadenitis)

• Rashes

• Vaginal dryness

• Chronic cough or shortness of breath

• Weakness, numbness, burning, or tingling in the extremities 

Which tests help diagnose Sjögren’s?

If your symptoms and/or physical exam raise concern for Sjögren’s, your provider will order more tests to confirm the diagnosis.  

Additional tests might include:

• Blood tests: Examples include antinuclear antibody (ANA), anti-Ro (or anti-SS-A), anti-La (or anti-SS-B), and rheumatoid factor (RF).

• Tests to confirm dry eye: An ophthalmologist (eye specialist) often does these.

• Tests to confirm dry mouth: These are to measure how much saliva you make.

• Tests to confirm immune cells are causing inflammation in the salivary glands: Examples include imaging with ultrasound or lip biopsy. A lip biopsy is a minor procedure that involves removing some of the tiny salivary glands and examining them under a microscope. 

Is Sjögren’s syndrome hard to diagnose?

Yes, diagnosing Sjögren’s disease can be tricky. A lot of things — like other medical conditions or medications — can cause dry eyes and dry mouth. Most of those things are far more common than Sjögren’s, so it’s important to rule them out.

Also, there is no single test that can confirm the diagnosis. It can take some time and effort to get through the testing, which can be frustrating.

How is Sjögren’s different from other immune system disorders?

Dry eyes and dry mouth are generally what make SJD different from other autoimmune conditions. But it’s possible to have dry eyes and dry mouth with other autoimmune conditions, like rheumatoid arthritis or lupus. This is especially true if you have positive anti-Ro antibodies. 

And many of the other symptoms of Sjögren’s disease overlap with other autoimmune disorders. This is another reason why it’s often difficult to diagnose them.

How do providers monitor Sjögren’s disease?

At regular appointments, your provider will ask about new or worsening symptoms and perform a physical exam. They’ll order blood and urine tests to make sure your organs are functioning properly. 

To monitor for lung problems due to Sjögren’s disease, your provider might order any of these:

• Chest X-ray

• Pulmonary function tests, which measure how well air moves in and out of the lungs 

• CT scan of the chest, which is only necessary if you have symptoms like cough or shortness of breath

Pregnancy

If you become pregnant and your blood tests are positive for anti-Ro antibodies, your obstetrician will perform ultrasounds of the baby more often than usual. That’s because anti-Ro antibodies cause a 2% risk of complete heart block, which prevents the baby’s heart from beating normally. People without anti-Ro antibodies aren’t at risk of this complication.

Lymphoma

People with Sjögren’s disease also have a 10- to 44-fold increased risk of non-Hodgkin’s lymphoma (NHL), a type of blood cancer. That’s because NHL is cancer of lymphocytes — the same immune cells that cause inflammation in the salivary glands in Sjögren’s. This may sound scary, but NHL is very treatable if caught early. 

To screen for lymphoma, your provider may order regular blood and urine tests. If your salivary glands remain swollen for several months or are rapidly getting bigger, your provider might recommend a biopsy. 

Lymphoma in the salivary glands causes persistent swelling because there are too many cancerous immune cells invading the gland. A biopsy is a minor procedure that involves removing a small piece of the gland and looking for cancerous lymphocytes under a microscope.

How do you treat Sjögren’s disease?

Sjögren’s is different for every person who has it. Treatment depends on your specific symptoms. Many lifestyle changes, over-the-counter medications, and prescription medications can help treat dry eye and dry mouth. Sometimes, no treatment is necessary.

A new diagnosis of Sjögren’s can be a lot to process. But there are many good resources to help cope. Together with your provider, you’ll figure out the best way to keep you as healthy as possible.

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Written by:

Samantha C. Shapiro, MD

Samantha Shapiro, MD, is a board-certified rheumatologist and internist with expertise in autoimmune and inflammatory conditions. She founded the division of rheumatology at Dell Medical School at The University of Texas at Austin.

Edited by:

Mandy Armitage, MD

Mandy Armitage, MD, has combined her interests in clinical medicine with her passion for education and content development for many years. She served as medical director for the health technology companies HealthLoop (now Get Well) and Doximity.

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