What Is Sjrögen's Syndrome? | Causes | How Sjrögen's and Lymphoma Are Connected | Signs and Symptoms | Get Support

If you have been diagnosed with Sjögren’s (pronounced SHOW-grins) syndrome, your health care 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 are connected.

Although people diagnosed with Sjögren’s syndrome have an increased risk of developing malignant lymphoma, the chances are still very small. Here’s what you need to know about the connections between these conditions.

What Is Sjögren’s Syndrome?

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 Sjögren’s syndrome are dry mouth and dry eyes. However, Sjögren’s syndrome can also target other areas of the body, potentially causing vaginal dryness, swollen salivary glands, dry skin, muscle and/or joint pain, and exhaustion.

What Causes Sjögren’s Syndrome?

No one knows exactly what causes or triggers Sjögren’s syndrome. 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.

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

The Connection Between Sjögren’s Syndrome and Lymphoma

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

If you are diagnosed with Sjögren’s syndrome, your doctor will most likely tell you about the potential risk of developing NHL. They will explain the symptoms of NHL and ask you to keep an eye out for them. This is important, as NHL is often curable when found early.

If you have Sjögren’s syndrome, you may have:

• Night sweats
• Unintentional weight loss
• Swollen (but painless) glands, often in the neck, armpit, or groin areas

In early 2020, researchers found that having a diagnosis of non-Hodgkin lymphoma may also predispose a person to develop Sjögren’s syndrome. The risk of developing one condition after being diagnosed with the other appears to be the highest in the initial year after the first diagnosis. An earlier study supported this conclusion, as it indicated that some people in the study developed lymphoma before they were diagnosed with Sjögren’s syndrome.

Because this study is so recent, more research is necessary to determine how Sjögren’s syndrome and NHL are connected.

Which Signs and Symptoms of Sjögren’s Syndrome Predict the Development of Lymphoma?

Researchers are not yet sure how Sjögren’s syndrome and NHL are connected. They have found that certain factors — including swollen salivary glands and Raynaud’s phenomenon — seem to predispose a person with Sjögren’s to develop NHL as well. Many of these factors can be identified using blood testing.

Having any one of the following risk factors alone does not necessarily mean that you have a higher risk of developing lymphoma. Researchers have determined the risks as follows:

• People with two or fewer of these signs and symptoms have a 3.8 percent chance of developing the condition.
• Those with three to six risk factors have a 39.9 percent chance of developing lymphoma.
• People with all seven risk factors have nearly a 100 percent chance of being diagnosed with lymphoma at some point.

Salivary Gland Enlargement

Some people diagnosed with Sjögren’s find that their salivary glands swell significantly. According to one study, people with Sjögren’s syndrome with lymphoma most often developed cancer in their salivary glands.

Enlarged salivary glands can be painful and may make it difficult to move the jaw while talking or eating. This swelling most often occurs in the parotid glands, which are found behind the jaw near the ears. However, it can also occur in the salivary glands located under the tongue and the jaw.

Lymphadenopathy

Lymphadenopathy is a condition in which the lymph nodes swell or enlarge and can occur in people with Sjögren’s. People often have small, soft lumps that may be tender or painful to the touch. Painless, swollen lymph nodes are often the first sign of lymphoma.

Raynaud’s Phenomenon

Raynaud’s phenomenon, also referred to as Raynaud’s disease, is an autoimmune condition sometimes associated with Sjögren’s. It causes a reduction of blood flow to the fingers and/or toes and is often associated with exposure to cold temperatures or with significant to extreme stress.

Anti-Ro/SSA and/or Anti-La/SSB Positivity

Anti-Ro/SSA and anti-LA/SSB are antibodies that are often (but not always) present in people with systemic (body-wide) autoimmune diseases, including Sjögren’s syndrome.

Rheumatoid Factor Positivity

Rheumatoid factor is associated with several autoimmune conditions — most commonly rheumatoid arthritis. In people with Sjögren’s syndrome, rheumatoid factor has also been found to be a predictor of lymphoma.

Monoclonal Gammopathies

Monoclonal gammopathies refer to a set of conditions in which abnormalities are present in some of the antibodies in the bloodstream. One type of monoclonal gammopathy, known as mixed monoclonal cryoglobulinemia, predicts whether lymphoma will develop in people with primary Sjögren’s syndrome.

Complement Component 4 Hypocomplementemia

Complement component 4 is a blood test in which doctors look at how certain proteins that aid the immune system are working in your body. When a person does not have enough of these proteins, it is called complement component 4 hypocomplementemia and is an independent predictor of developing NHL.

Other Symptoms of Sjögren’s Syndrome

Several other symptoms of Sjögren’s syndrome may predict the development of lymphoma:

• Purpura (red/purple spots) on the body
• Tongue atrophy, in which the tongue’s texture changes and it may seem to get smaller
• Cryoglobulinemia (a type of vasculitis that causes inflamed blood vessels)
• B-cell activation in the immune system

Your health care team may choose to assess you for these factors depending on the other symptoms you’re having or the risk factors you develop.

Doctors are now using computer models to help determine a person’s risk of lymphoma after a diagnosis of Sjögren’s syndrome. If you are concerned about your health or that of a loved one with this diagnosis, talk to your doctor today. Ask how you may be able to access this tool or something similar.

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