EPIDEMIOLOGY AND HEALTH-RELATED SERVICES: Edited by Allan C. GelberIncidence of lymphoma associated with underlying lupus lessons learned from observational studiesBichile, Tanmayee; Petri, MichelleAuthor Information Johns Hopkins University School of Medicine, Division of Rheumatology, Baltimore, Maryland, USA Correspondence to Michelle Petri, MD, MPH, Johns Hopkins University School of Medicine, 1830 E Monument Street, Suite 7500, Baltimore, MD 21205, USA. Tel: +1 410 955 9114; e-mail: [email protected] Current Opinion in Rheumatology: March 2014 - Volume 26 - Issue 2 - p 111-117 doi: 10.1097/BOR.0000000000000026 Buy Metrics Abstract Purpose of review 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. Recent findings 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. Summary Although the association of non-Hodgkin's lymphoma in SLE is well established, risk factors attributing to this association are still not understood. © 2014 Lippincott Williams & Wilkins, Inc.