The over-activation of B cells is one of the typical features of pSS, which is clinically manifested as hypergammaglobulinemia, the production of various autoantibodies, high levels of B-cell-activating factor, B-cell subset disorders and a significant increase in the risk of lymphoma, with approximately 5% of patients with pSS eventually progressing to lymphoma (11). This evidence concerns the gene TNFSF13B and lymphoma.