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International Journal of Rheumatology
Volume 2009 (2009), Article ID 424935, 8 pages
Review Article

Controversies on Rituximab Therapy in Sjögren Syndrome-Associated Lymphoproliferation

1Rheumatology Clinic, DPMSC, Azienda Ospedaliero Universitaria “S. Maria della Misericordia”, University of Udine, 33100 Udine, Italy
2U.O.C. di Medicina, Presidio Ospedaliero di San Daniele del Friuli, San Daniele del Friuli, 33038 Udine, Italy

Received 28 February 2009; Accepted 15 December 2009

Academic Editor: Debashish Danda

Copyright © 2009 Luca Quartuccio et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by chronic inflammation of salivary and lachrymal glands, and frequently accompanied by systemic symptoms. A subgroup of SS patients develops malignant B cell non-Hodgkin's lymphoma (NHL), usually of the mucosa-associated lymphoid tissue (MALT) type and very often located in the major salivary glands. Currently, there is a lack of evidence-based intervention therapy which may influence SS-related chronic inflammation and lymphoproliferation. B cells are involved in the pathogenesis of SS, and B cell downregulation may lead to a decrease of disease activity. Rituximab (RTX), a chimeric monoclonal antibody targeting the CD20 antigen on the B cell surface, has been successfully investigated in other autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematosus, ANCA-associated vasculitis, and mixed cryoglobulinemic syndrome. Preliminary experiences of RTX therapy in SS patients with or without a lymphoproliferative disorder suggest that SS patients with more residual exocrine gland function might better benefit from RTX. Efficacy of RTX in SS-associated B-cell lymphoma, mainly in low-grade salivary gland lymphomas, remains an open issue.