[Sjögren's syndrome and non-Hodgkin's lymphoma: from pathophysiology to treatment]

Abstract

Although infrequent, the development of non-Hodgkin's lymphoma (NHL) during the course of Sjögren's syndrome represents a major complication. Nethertheless, most NHL developing in patients with Sjögren's syndrome are B cell lymphomas involving the marginal zone, localized to extranodal sites. Predictive factors include lymphadenopathy, splenomegaly, neutropenia, cryoglobulinemia and low C4 levels. The treatment of B cell lymphomas depends on its nature and localization. Biotherapies targeting the B lymphocyte, such as rituximab, a chimeric monoclonal anti-CD20 antibody, or more recently targeting BAFF can be proposed.