Treatment Algorithm for the Prevention of End-Stage Renal Disease in Children with Lupus Nephritis

Abstract

Chronic kidney disease is one of the most common complication of systemic lupus erythematosus, which if untreated can lead to  the end-stage renal disease (ESRD). Early diagnosis and adequate treatment of lupus nephritis (LN) is critical to prevent the chronic kidney disease incidence and to reduce the development of ESRD. The treatment of LN has changed significantly over the past decade. In patients with active proliferative LN (Classes III and IV) intravenous methylprednisolone 1 g/m2/day for 1-3 days then prednisone 0.5-1.0 mg/kg/day, tapered to <0.5 mg/kg/day after 10-12 weeks of treatment plus mycophenolate mofetile (MMF)
1.2 g/m2/day for 6 months followed by maintenance lower doses of MMF 1-2 g/day or azathioprine (AZA) 2 mg/kg/day for 3 years have proven to be efficacy and less toxic than cyclophosphamide (CYC) therapy. Patients with membranous LN (Class V) plus diffuse or local proliferative LN (Class III and Class IV) should receive either the standard 6 monthly pulses of CYC (0.5-1 g/m2/month) then every 3
rd month or to a shorter treatment course consisting
of 0.5 g/m2
IV CYC every 2 weeks for six doses (total dose 3 g)
followed by maintenance therapy with daily AZA (2mg/kg/day) or MMF (0.6 g/m
2/day) for 3 years. Combination of MMF plus
rituximab or MMF plus calcineurin inhibitors may be an effective co-therapy for those refractory to induction or maintenance therapies. This report introduces a new treatment algorithm to prevent the development of ESRD in children with LN.

Keywords:End-stage renal disease, lupus, nephritis, treatment algorithm