Effect of low-dose cyclosporine A in the treatment of refractory proteinuria in childhood-onset lupus nephritis

We performed a prospective study to evaluate the efficacy and safety of low-dose cyclosporine A (CSA) treatment in paediatric lupus nephritis refractory to conventional therapy. Seven children with biopsy-proven Class III-IV lupus nephritis were treated with CSA (2-4 mg/kg/day) combined with low-dose prednisone for one year. All patients had failed to achieve sustained proteinuria remission with corticosteroids and cytotoxic drugs. Proteinuria decreased from median value of 2.5 g/24 hours (range, 1.2-4.9) to 0.14g/24hours (range, 0.0-0.84) after treatment (P = 0.018). Median values of creatinine clearance and serum creatinine did not change significantly. Median systemic lupus erythematosus disease activity index score decreased from 12 (range, 6-16) to 4 (range, 0-8) at end of treatment (P = 0.027). However, two patients experienced flares of extrarrenal manifestations and complement levels did not improve. Moreover, most patients relapsed with proteinuria within a few months of stopping CSA therapy. Side effects were not significant. In conclusion, low-dose of CSA combined with steroids appears to be useful to reduce proteinuria in paediatric proliferative lupus nephritis refractory to steroids and cytotoxic drugs; however, relapses are common after CSA discontinuation. Further studies are needed to define the precise role of CSA in paediatric lupus nephritis.