Catarina Dias and David A. Isenberg
Abstract
Autoimmune rheumatic diseases (ARD), such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and Sjögren’s syndrome (SS), have consistently been associated with the development of B-cell non-Hodgkin lymphoma (BCNHL). In this Review, we focus on reports published since 2006 and summarize the data regarding the BCNHL subtypes and clinical findings associated with this increased risk.
Patients with these ARD, particularly those with detectable autoantibodies and systemic involvement, are at increased risk of developing BCNHL, especially diffuse large B-cell lymphoma and marginal zone lymphoma.
SS shows the strongest association with BCNHL. Male sex, advanced age, prolonged disease course and increased disease severity, but not family history of autoimmune conditions, seem to be associated with an increased risk of non-Hodgkin lymphoma. Chronic immune stimulation, genetic and environmental factors and some immunosuppressive drugs might be involved in lymphomagenesis in these patients. The reason why some ARD are associated with BCNHL and other autoimmune diseases are not remains unclear. These associations are important as they provide information about the mechanisms of lymphomagenesis, and might help identify new therapeutic targets.