What are risk factors for lymphoma development in Sjogrens Syndrome?
The list of risk factors continues to grow. Patients with persistent salivary gland enlargement are at highest risk. Other clinical risk factors include lymphadenopathy, Raynaud’s phenomenon, splenomegaly, palpable purpura, glomerulonephritis, peripheral neuropathy, disease duration (>10 years), and moderate-high disease activity as measured by the EULAR SS Disease Activity Index >5. Laboratory and histologic features associated with lymphoma include monoclonal gammopathy (IgM kappa most common), loss of a previously positive RF, leukopenia, cryoglobulinemia, low C4 or C3, and the presence of germinal centers or high FS (≥3) on the initial MSG biopsy.
Experts recommend blood tests (complete blood count, protein electrophoresis, RF, C3 and C4, and possibly cryoglobulins) every 1 to 2 years to monitor risk. If lymphoma is suspected, the evaluation should proceed with imaging (e.g., MRI or positron emission tomography) and tissue biopsy to confirm diagnosis.