If you’ve been diagnosed with Sjögren’s (pronounced SHOW-grins) disease, your healthcare provider may have asked you to also watch for symptoms of lymphoma. These cautionary words can certainly be scary, so it’s important to understand both conditions and how they’re connected.

Although people diagnosed with Sjögren’s disease have an increased risk of developing malignant lymphoma, the chances are still very small. Similarly, having lymphoma may also raise your risk of developing Sjӧgren’s, though more research is needed to determine whether this is true and, if it is, how it works. Here’s what you need to know about the connections between these conditions.

What Is Sjögren’s Syndrome?

Sjӧgren’s disease — formerly known as Sjögren’s syndrome — is an autoimmune disease. It occurs when the immune system mistakenly attacks the body’s healthy tissues. It most commonly targets the parts of the body responsible for producing fluids, such as saliva and tears.

The characteristic symptoms of Sjӧgren’s disease are dry mouth and dry eyes. However, Sjögren’s disease can also target other areas of the body, potentially causing sore throat or dry cough, vaginal dryness, swollen salivary glands, dry skin, skin lesions, muscle and/or joint pain, and fatigue (tiredness).

What Causes Sjögren’s Syndrome?

No one knows exactly what causes or triggers Sjögren’s disease. There seems to be a genetic predisposition, as those with certain genes seem more likely to develop the condition. However, it also appears that there must be a specific trigger — such as infection with certain viruses or bacteria — before the syndrome develops.

According to Mayo Clinic, Sjӧgren’s disease is usually diagnosed in women over the age of 40, many of whom have also been diagnosed with other rheumatic diseases such as rheumatoid arthritis or lupus. It can occur by itself (which is called primary Sjӧgren’s) or alongside other autoimmune conditions (which is called secondary Sjӧgren’s).

Does Sjögren’s Cause Lymphoma?

Research has found that people diagnosed with Sjӧgren’s disease are significantly more likely than those in the general population to develop non-Hodgkin lymphoma (NHL) — a type of blood cancer that originates in white blood cells called lymphocytes. Despite this connection, the chances of developing NHL with Sjӧgren’s disease are still minimal.

Sjӧgren’s disease may also be connected to other types of lymphoma, though additional research is needed to determine whether this is true and what the actual connection is.

If you’re diagnosed with Sjӧgren’s disease, your rheumatology team might inform you about the potential of a higher risk of lymphoma. It’s important to know the symptoms of NHL so you can keep an eye out for them. Many types of NHL are curable when found early.

Symptoms of NHL may include:

• Night sweats
• Unintentional weight loss
• Swollen glands, often in the neck, armpit, or groin areas
• Unexplained fevers
• Chest or belly pain, swelling, or enlargement, which may cause coughing or problems breathing

If you’re living with Sjӧgren’s disease and you notice any of these symptoms, talk to your rheumatologist right away. This is particularly true if you have Sjӧgren’s and very swollen lymph nodes. Your doctor can help you find the cause of the symptoms, whether it’s lymphoma or an unrelated condition, and start proper treatment.

Can Lymphoma Cause Sjögren’s Disease?

There’s some question as to whether being diagnosed with non-Hodgkin lymphoma can also make it more likely that you’ll develop Sjӧgren’s disease. In 2020, researchers in Taiwan looked at more than 25,000 people diagnosed with NHL and found that they were more likely than the general population to develop Sjӧgren’s disease. However, out of those 25,000-plus people, only 49 developed Sjӧgren’s.

It’s important to note that other researchers have questioned the methodology used in the 2020 study. The study didn’t do a good job of determining whether the people who developed Sjӧgren’s had been diagnosed with any other autoimmune diseases. These could have been the connection to Sjӧgren’s, rather than NHL.

Critics also questioned whether the diagnosis of Sjӧgren’s had not come first for at least some of the people, based on how people qualified for the study. Finally, the study didn’t ask whether the Sjӧgren’s that developed in those 49 people may have been caused by treatments for NHL, rather than NHL itself.

Other researchers asked additional questions about how the original study was done. Additionally, no research done on the topic since 2020 has come up with results similar to those of the 2020 study.

None of this proves that NHL doesn’t raise the risk for developing Sjӧgren’s later. It does mean that we don’t have enough evidence to confirm this relationship and that more research on the conditions is necessary to learn more.

What Predicts Lymphoma in Sjögren’s?

Researchers believe that the connection between Sjӧgren’s disease and lymphoma centers around cells in your body called B lymphocytes, or B cells. These cells produce proteins called antibodies that are an active part of your immune system. They can bind to invaders, like toxins, bacteria, or viruses, to prevent them from harming your body. They can also call other immune cells to destroy a cell in your body that has already been invaded.

In people living with Sjӧgren’s disease, these B lymphocytes are too active. They build up in the glands that create your saliva and in other places, causing an inflammatory response that results in Sjӧgren’s disease.

Non-Hodgkin lymphoma most often starts in B lymphocytes. Constantly stimulating B lymphocytes to grow and be more active than they need to be can set you up to develop the abnormalities in them that lead to B-cell lymphoma, a type of NHL.

There are several clinical findings of Sjӧgren’s disease that may make it more likely you’ll develop NHL. Common clinical findings that are associated with a higher risk of developing lymphoma include:

• Persistent enlargement of the salivary glands
• Enlargement of the lymph nodes
• The appearance of cryoglobulinemic vasculitis (a form of inflammation of the blood vessels)

If you experience one of these symptoms, you should contact your doctor.

There are also various laboratory findings that may be associated with a higher risk of developing lymphoma. These include detection of:

• Too many antibodies in your bloodstream
• Rheumatoid factor (an antibody your immune system can make by mistake)
• Cryoglobulins (a kind of protein in your blood)
• Monoclonal gammopathy (an increased formation of immunoglobulins)
• A low count of lymphocytes
• Low complement components (such as the proteins C3 and C4)

If you have Sjögren’s disease, these laboratory findings need to be verified regularly, especially if you have had the diagnosis for more than five years, as the risk of developing a malignancy is associated with the period of time that has passed since the beginning of the disease. If your doctor is concerned that you might be developing lymphoma, they can order blood tests to see if you have any of these signs.

Talk to Your Doctor

If you have been diagnosed with Sjögren’s disease and you’re concerned about developing lymphoma, talk to your healthcare team. They can help you assess your personal risk factors and help you decide if further testing might be a good idea.

If you feel particularly worried about developing lymphoma, your doctor can also follow up and connect you to resources in your area that will support your mental health. Knowing you have a higher chance of getting cancer can be scary, and a trained therapist or counselor can help you process those feelings while monitoring your symptoms.

If you’ve noticed symptoms that may indicate NHL, talk to your doctor as soon as possible. The sooner you get blood tests to check for NHL, the sooner you’ll know. While very few people with Sjӧgren’s do develop NHL, if you have it, starting treatment early will better your chances of beating blood cancer.

Meet Others Who Understand

MyLymphomaTeam is the social network for people with lymphoma. On MyLymphomaTeam, more than 21,000 members come together to ask questions, give advice, and share their stories with others who understand life with lymphoma.

Have you experienced Sjögren’s disease alongside lymphoma? Which diagnosis did you receive first? Share your experience in the comments below, or start a discussion by posting on your Activities page.