Revised: August 28, 2013
Accepted: March 3, 2014
Published online: May 6, 2014
Childhood-onset systemic lupus erythematosus (cSLE) is a severe multisystem autoimmune disease. Renal involvement occurs in the majority of cSLE patients and is often fatal. Renal biopsy is an important investigation in the management of lupus nephritis. Treatment of renal lupus consists of an induction phase and maintenance phase. Treatment of childhood lupus nephritis using steroids is associated with poor outcome and excess side-effects. The addition of cyclophosphamide to the treatment schedule has improved disease control. In view of treatment failure using these drugs and a tendency for non-adherence, many newer agents such as immune-modulators and monoclonal antibodies are being tried in patients with cSLE. Trials of these novel agents in the pediatric population are still lacking making a consensus in the management protocol of pediatric lupus nephritis difficult.
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.