One of the most serious potential complications of Sjögren’s disease is the development of lymphoma and other cancers, particularly blood cancers. People with Sjögren’s have a significantly higher risk of non-Hodgkin lymphoma (NHL) compared to the general population, with B-cell lymphomas being the most common. Fortunately, many lymphomas associated with Sjögren’s tend to grow slowly, allowing for careful monitoring and timely treatment when needed.
The exact reason Sjögren’s increases cancer risk is not fully understood, but it is believed to be linked to chronic immune system activation and long-standing inflammation. Continuous stimulation of B cells, impaired regulation of cell death (apoptosis), and immune system dysfunction all contribute to an environment where malignant cells are more likely to develop. Certain clinical features and disease characteristics can signal a higher likelihood of lymphoma, reinforcing the importance of regular medical follow-up and vigilance.
The most frequent lymphoma seen in Sjögren’s is B-cell MALT (mucosa-associated lymphoid tissue) lymphoma, which commonly affects the salivary glands but can also involve other mucosal tissues. Other types of lymphomas and some solid tumors—such as thyroid, lung, stomach, breast, and colorectal cancers—have also been reported at increased rates. In addition, some blood cancers, including leukemia and multiple myeloma, occur more often in people with Sjögren’s than in the general population.
Prognosis varies depending on the type and aggressiveness of the cancer. Many Sjögren’s-related lymphomas are indolent and may be managed with a “watch and wait” approach, while more aggressive forms require prompt treatment. Symptoms such as unexplained fevers, night sweats, weight loss, or rapidly enlarging lymph nodes warrant immediate medical evaluation.
Treatment decisions aim to balance controlling Sjögren’s disease activity with minimizing cancer risk. Managing inflammation and immune overactivity is essential, as uncontrolled disease itself may increase malignancy risk. While some immunosuppressive and biologic therapies are necessary to manage severe Sjögren’s, their use requires careful consideration, ongoing monitoring, and individualized risk–benefit discussions between patients and healthcare providers.
Overall, lymphoma—especially B-cell MALT lymphoma—is the most common malignancy associated with Sjögren’s. Awareness of risk factors, regular monitoring, early recognition of warning signs, and advances in targeted therapies have improved outcomes. With appropriate surveillance and management, many patients with Sjögren’s who develop lymphoma can be treated effectively and achieve good long-term outcomes.
*summary courtesy of The Sjögren's Book, Fifth Edition, Daniel J. Wallace, MD
