Revised: August 28, 2013
Accepted: March 3, 2014
Published online: May 6, 2014
Core tip: Childhood-onset systemic lupus erythematosus (cSLE) is a rare but severe autoimmune disease with multisystem involvement. Renal disease occurs in 50% to 75% of all cSLE patients and is a major cause of increased morbidity and mortality. Originally SLE nephritis was treated with steroids with a poor outcome which improved markedly with the introduction of cyclophosphamide, but at the cost of increased side effects which resulted in a further search for a less toxic, but equally effective regime. Here we discuss some newer drugs including immune-modulators and monoclonal antibodies in addition to azathioprine and mycophenolate mofetil, however, most of the evidence on these medications is restricted to adult literature and pediatric data are extrapolated from these trials.