Abstract
Renal involvement occurs in 50-75% of children with childhood-onset systemic lupus erythematosus (cSLE). Proliferative lupus nephritis (LN) represents the most common pattern of renal involvement in cSLE. Despite aggressive treatment, progression to end stage renal disease can occur in up to 5-10% of children. Over the last 2 decades, tremendous advancements have been made in the treatment of pediatric LN. Special considerations in children need to address the impact of disease and therapy on both physical and psychological growth and development. This review will focus on pivotal clinical trials in the treatment of proliferative LN, with a focus on pediatric data when available.
Highlights
Systemic Lupus Erythematosus (SLE) is a chronic, autoimmune disease characterized by multi-organ system involvement, widespread inflammation and the presence of autoantibodies
Despite the similarities between Childhood-onset SLE (cSLE) and adult-onset disease, children have a higher frequency of major organ involvement and are more likely to suffer from damage accrual throughout their lifespan
This review will focus on pivotal clinical trials in the treatment of proliferative lupus nephritis (LN), with a focus on pediatric data when available
Summary
Systemic Lupus Erythematosus (SLE) is a chronic, autoimmune disease characterized by multi-organ system involvement, widespread inflammation and the presence of autoantibodies. In a Dutch Lupus Nephritis Study, patients treated with oral AZA (2 mg/kg/day) and corticosteroids as induction therapy for proliferative LN were more likely to have renal relapse and increasing serum creatinine as compared to intravenous CYC in long-term follow up[12,24,25]. In a sub-analysis of adolescent patients (12–18 years old) who participated in the ALMS study, results were similar to that of the adults with equivocal renal response rates between MMF and intravenous CYC for induction therapy and comparable efficacy between MMF and AZA for the maintenance phase[41]. In another study targeted toward the maintenance phase of therapy, cyclosporine A was shown to be as effective as AZA after induction with oral CYC55 This data, promising, had limited follow up and has been insufficient to support the use of tacrolimus or cyclosporine A as initial therapeutic agents in management of proliferative LN. One group has piloted the “steroid- avoiding rituxilup” protocol consisting of two doses of rituximab (1 g/dose) and intravenous methylprednisolone (500 mg) followed by maintenance therapy
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