We found a higher risk of MALT lymphoma in patients presenting with systemic activity, positive cryoglobulins, and low C3 levels at SjS diagnosis, while the risk of non-MALT B-cell lymphomas was unrelated to systemic activity, with anemia, monoclonal gammopathy, cryoglobulins, and low C4 levels at SjS diagnosis being the main risk factors. This evidence concerns the gene C3 and Schwartz-Jampel syndrome.