Systemic lupus erythematosus and lymphoma: Incidence, pathogenesis and biology

Systemic Lupus Erythematosus (SLE), a well recognized systemic autoimmune disease is associated with an increased risk of malignancies, particularly lymphoma. Various studies have shown this risk to be as high as 4-7-fold compared to the general population. The pathogenesis of lymphoma in patients with SLE is still not well understood. In this review we summarize the world literature and update current knowledge on the interesting link between SLE and lymphomagenesis. We relate in turn to incidence rates of lymphoma in SLE and subtypes of lymphoma encountered; pathogenesis and relevant theories proposed; links with EBV and the possible role of continued activity of lupus and of immunosuppressive therapy in lymphomagenesis. It is clearly evident that further studies are needed to improve the understanding of this association. Some cytokines and proteins associated with cell survival and proliferation, such as BAFF, APRIL, IL6 and BCL2, have been found to be elevated both in SLE and lymphoma. These factors may well impact pathogenesis, however, a direct "cause and effect" relationship is yet to be demonstrated.