Incidence of lymphoma associated with underlying lupus: lessons learned from observational studies

The increased risk of overall malignancy in systemic lupus erythematosus (SLE) is well established. Cohort studies have shown a significant association of non-Hodgkin's lymphoma with SLE. This review will focus on the associated risk factors, the role of immunology, immunosuppressive medications and secondary Sjogren's syndrome in the development of lymphoma in SLE.

Cohort studies have established the link between non-Hodgkin's lymphoma and SLE. The largest study to date has been of 16 409 patients from 30 rheumatologic centres. In the last decade, insights into the roles of BAFF (BLyS) and APRIL in lymphoma development have helped to understand the mechanism leading to monoclonality. Genetic markers have helped to prognosticate diffuse large B-cell lymphoma. The contribution of disease activity and immunosuppressive drugs in lymphoma development is still not understood in SLE.

Although the association of non-Hodgkin's lymphoma in SLE is well established, risk factors attributing to this association are still not understood.