Given that TNFAIP3 is considered a gatekeeper of the abnormal activation of the NF-κB pathway, the increased frequency of the TNFAIP3 functional variant in SS patients complicated by lymphoma [12, 32] along with the previously reported lower levels of the A20 protein in MSG tissues derived from SS patients complicated by MALT [11] support the idea of A20-related deregulation of NF-κB pathways in SS-related lymphomagenesis. This evidence concerns the gene TNFAIP3 and lymphoma.