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LYMPHOMA & SJOGREN'S
Key Concepts
Non-Hodgkin lymphoma (NHL) affects 5-10% of Sjogren's patients over a lifetime. Patients have very different risk levels, which can be determined by monitoring for risk factors as part of ongoing care.
This page reviews a recent model developed to predict the risk of developing lymphoma in individual Sjogren's patients (294).
This page does not cover the clinical evaluation and care of lymphoma. For an excellent review of those topics, see the Sjogren's Foundation page on Lymphoma and Sjogren's Disease.
This page is for educational purposes only. It is not an official guideline, and does not constitute medical advice.
Understanding Lymphoma and Risk Factors
Lymphoma is a cancer of the immune system.
In Sjogren's, it usually develops when B cells (the white blood cells that make antibodies) multiply out of control due to ongoing immune system overactivity. Although lymphoma is one of the most serious systemic complications of Sjogren's, most patients respond well to treatment, with an overall survival rate of 92% at 5 years (295).
This page explains a simple scoring tool that checks 7 risk factors for lymphoma. It is meant to be used at diagnosis and repeated over time. If your risk is high, your doctor may recommend more frequent visits and extra tests.
Think of risk factors as clues, not causes.
Having a risk factor does not mean it causes lymphoma; it simply means researchers have noticed they often show up together. For example, antibodies like anti-SSA, anti-SSB, or rheumatoid factor (RF) are linked to higher risk, even though those antibodies are unlikely to cause or promote lymphoma.
7 Independent Risk Factors For Lymphoma
Researchers developed a simple scoring system called the Athens Model to estimate the chance of developing lymphoma (294). You get 1 point for each of 7 key risk factors. Your doctor checks for the first 3 during a physical exam and looks for the last 4 with routine blood tests:
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Salivary gland enlargement
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Lymphadenopathy (enlarged lymph nodes)
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Raynaud’s phenomenon
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Anti-SSA or anti-SSB
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Rheumatoid factor (RF)
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Monoclonal gammopathy (detected via SPEP blood test)
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Low C4 (a type of complement)
This scoring system does not tell you whether you will or will not get lymphoma. Instead, it gives you an idea of your risk level by identifying how many of the 7 risk factors you have. The probability of NHL development in this model was 3.8% (about 4 in 100) for patients with 0-2 risk factors, which is roughly double the rate in the general population. The risk of developing lymphoma continues to rise with more risk factors.
Important Context: This tool was developed based on data from 381 Sjogren's patients at a tertiary (specialized) medical center in Athens, Greece. It may not reflect the general patient population, which is likely to include more lower-risk patients.
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A high score is not a reason to panic, just a reminder to talk with your doctor about closer monitoring. Everyone is different, and many people with high scores (5-7 risk factors) never develop lymphoma.
Additional Clinical Predictors
Other models have been developed, but the Athens model is a strong starting point because it combines readily available clinical and serologic (blood test) predictors into a simple additive risk estimate.
Additional risk factors are also associated with lymphoma and can be helpful when considering the entire clinical picture:
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Cryoglobulinemia or cryoglobulinemic vasculitis
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High ESSDAI score (a research tool that measures a subset of systemic manifestations)
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High focus score on a lip biopsy
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Low white blood cell counts (lymphopenia)
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​Communication Tips: Talking About Risk Factors With Your Rheumatologist
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Blood Tests
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SSA, SSB, and RF: Most patients have already had tests for anti-SSA, anti-SSB, and RF. If yours were negative, it is good to repeat them on occasion. However, SSA and SSB do not need to be rechecked routinely once, they are positive. Once positive, you should always be considered SSA or SSB positive.
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Complement and SPEP: Ask your doctor about blood tests for complement (C3 and C4) and serum protein electrophoresis (SPEP). These are often checked once a year. While C3 and C4 are usually ordered together, a low C4 level is the key marker linked to lymphoma risk.
Physical Findings - Be sure to point out the following symptoms or changes during your exams:
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Enlarged lymph nodes: Particularly in the neck or head, including the face.
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Enlarged salivary glands or salivary gland masses: Including the parotid glands (in the cheeks) or the submandibular glands (under the jaw).
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Raynaud's phenomenon: If you develop Raynaud's phenomenon, take photos of the color changes in your fingers or toes to share with your doctor.
General Symptoms
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See the Sjogren's Foundation Lymphoma and Sjogren's Disease page for information about general signs and symptoms of lymphoma, which vary widely between patients. Some patients have no obvious symptoms.
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Note on fatigue: Increased fatigue is common with lymphoma, but it also has many other causes in Sjogren's. See Fatigue Evaluation and Treatment for more information.
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Key Links and Self-Advocacy Tools
Updated 11-14-2026