Research so far has revealed a lot about Sjögren’s disease, previously called Sjögren’s syndrome. Doctors and researchers know this condition is an autoimmune disease that causes dryness in areas like the eyes and mouth, and it can also affect other parts of the body in unexpected ways. However, the exact causes of Sjögren’s disease are still being studied.

Some people develop Sjögren’s on its own. Others have Sjögren’s along with another autoimmune disease, such as lupus or rheumatoid arthritis (RA). This is often called associated Sjögren’s (formerly known as secondary Sjögren’s). This article explores known and suspected risk factors for Sjögren’s disease.

How Does Sjögren’s Disease Start?

The causes of Sjögren’s disease aren’t fully understood, and researchers continue to study risk factors that may play a role in how it develops. They do know that Sjögren’s starts with an abnormal immune system response. Normally, the immune system protects the body from viruses and bacteria. In autoimmune disorders like Sjögren’s disease, the immune system mistakenly attacks healthy tissue as if it were a threat.

In Sjögren’s disease, the immune system mainly targets the glands that make tears and saliva. This leads to common Sjögren’s symptoms such as dry eyes, a gritty or sandy feeling in the eyes, and dry mouth. For many people, Sjögren’s affects other tissues and body parts too. This can lead to symptoms like dry skin, vaginal dryness, or a dry cough. If certain organs are involved, more serious symptoms such as shortness of breath, tingling in the hands or feet, skin rashes or sores, and joint pain may develop.

Researchers have linked certain genes to an increased risk of Sjögren’s disease. However, in many cases, these genes are triggered by another factor, such as a viral or bacterial infection or another autoimmune disease.

What Raises the Risk of Sjögren’s Disease?

Some factors don’t directly cause Sjögren’s disease, but they do increase the chance of developing it.

Being Female

Over 90 percent of people living with Sjögren’s disease are female, according to Cleveland Clinic. Researchers believe that the hormone estrogen may influence how the immune system works.

People who’ve gone through menopause seem to have a higher risk of Sjögren’s than those in the general population. During menopause, estrogen levels drop sharply. Because the risk of Sjögren’s rises around this time, some researchers think estrogen may help protect against the disease. Others believe the difference may be linked to genetics, since some Sjögren’s-related genes are found on the X chromosome. Typically, women have two X chromosomes and men have one, according to Johns Hopkins Medicine.

More research is needed to better understand these possible explanations.

Being Older

Age also appears to affect Sjögren’s risk. Although the disease can begin at any time, including during childhood or adolescence, most people are diagnosed between ages 45 and 55.

Having a Family History

Having an immediate family member with Sjögren’s disease, such as a biological parent or sibling, also raises your risk. One or both parents can pass on genes that increase the likelihood of developing the disease. Researchers estimate that genetics account for around 30 percent of the overall risk of Sjögren’s disease.

Family history alone usually isn’t a reason to get tested. Most healthcare providers recommend testing only if you have symptoms of an autoimmune condition.

You might opt for testing if you have a family member with Sjögren’s, have another autoimmune disease, and are planning a pregnancy. Certain blood tests can screen for autoantibodies linked to the disease that, in some cases, may affect a baby’s health. A doctor or rheumatologist can help you decide whether testing makes sense based on your symptoms and risk factors.

Having Another Rheumatic Disease

Autoimmune conditions often occur together. About half of people with Sjögren’s disease also have another autoimmune disease. People with rheumatic diseases have a particularly high risk of Sjögren’s.

Rheumatic diseases affect joints, muscles, and ligaments, but they can affect other organs too. There are more than 100 rheumatic diseases, including many types of arthritis. Some are autoimmune diseases. People with autoimmune rheumatic diseases, such as lupus and rheumatoid arthritis, are more likely to develop Sjögren’s disease.

Lupus can cause inflammation throughout the body, including in the joints. Joint pain and swelling can sometimes look similar to RA, but lupus may also cause symptoms like skin rashes, fever, and mouth sores during flares.

Rheumatoid arthritis develops when the immune system attacks the synovium — the tissue lining the inside of joints. RA often affects the same joints on both sides of the body, such as both elbows. Common symptoms include joint pain, swelling, and stiffness. Inflammation in small joints (such as in the hands) can sometimes affect other parts of the body too.

Other Possible Risk Factors

As researchers continue learning more about Sjögren’s, they have identified other possible risk factors and triggers.

Hormone Differences

Estrogen may play a role in Sjögren’s disease, but other hormones may be at work too. Researchers are looking into the possible influence of hormones like dehydroepiandrosterone (DHEA) and prolactin.

DHEA is made by the adrenal gland and is a building block for sex hormones, including estrogen. In the salivary glands, DHEA is converted to estrogen and another hormone called dihydrotestosterone. This process may be disrupted in people with Sjögren’s disease.

Compared with the general population, people with Sjögren’s are 1.3 to 2.4 times more likely to have hyperprolactinemia (high prolactin levels), which is also common in other autoimmune diseases. High prolactin levels don’t seem to be linked to disease severity, but some researchers believe prolactin may still play a role in Sjögren’s development.

Specific Genes

The genetic causes of Sjögren’s disease aren’t fully understood, partly because genetic risk factors appear to differ between populations. For example, genes linked to Sjögren’s in people of European descent may not be the same as the ones linked to the disease in people of Asian descent.

Researchers also continue to study epigenetic risk factors. Epigenetics looks at how behaviors and the environment can affect how genes work, without changing the genes themselves.

Environmental Triggers

In many cases, Sjögren’s disease appears to be triggered by infections, especially viral infections. No single virus has been identified as causing Sjögren’s, but researchers have noticed that many people diagnosed with the disease often had specific infections in the past. Viral infections that might raise the risk of Sjögren’s include:

• Epstein-Barr virus
• Hepatitis C
• Human T-lymphotropic virus 1
• Cytomegalovirus
• COVID-19

Researchers continue to explore the roles of other possible environmental risk factors for Sjögren’s disease, such as stressful life events and past smoking. These factors have been linked to other systemic autoimmune diseases, but more research is needed to confirm if factors like these raise the risk of Sjögren’s disease.

Talk With Your Doctor

Talk with your doctor or rheumatologist if you have possible symptoms of Sjögren’s disease, especially if you also have a rheumatic disease or other known risk factors. Your doctor can help determine if testing for Sjögren’s disease makes sense based on your symptoms and overall risk.

Join the Conversation

On MySjögrensTeam, people share their experiences with Sjögren’s disease, get advice, and find support from others who understand.

Which Sjögren’s risk factors do you have? Let others know in the comments below.