Specifically, those presenting with parotidomegaly, vasculitic purpura, splenomegaly, lymphadenopathy, cryoglobulinemia, low C4 levels, neutropenia and lymphopenia at SS diagnosis were seen to have a much greater probability of developing lymphoma and are hence considered as high-risk patients [1–4,6,8,13,14,19,32]. This evidence concerns the gene C4A and Splenomegaly.