Although more commonly presenting in adulthood, approximately 15-20% of systemic lupus erythematosus (SLE) cases occur before age 16 years. Unfortunately, SLE is usually more severe when presenting in childhood, and frequently involves vital organs such as the kidney. Over the past several decades, mortality rates have dropped, largely due to earlier diagnosis, improved management of the SLE and improved general medical care to reduce infection. Treatment strategies for nephritis in children is largely adopted from experience in adults, and the recent advances in therapeutic options for adults have brought new treatment to children. However, determining efficacy is difficult due to the absence of clinical trial data. Furthermore, determination of safety in a developing child or adolescent cannot be extrapolated from adult studies. As survival has improved, numerous secondary complications have emerged, including early atherosclerosis. As for adults with SLE, it is generally accepted that atherogenesis in SLE results from both disease- and treatment-related factors. Most surprising is that persons with childhood-onset SLE can develop myocardial ischaemia as early as 20-30 years of age. Better understanding of the pathogenesis and development of preventative strategies is needed to ensure that these young people do not succumb to atherosclerosis instead of to SLE. (C) 2009 Published by Elsevier Ltd.