Efficacy of methylprednisolone pulse therapy in steroid-resistant nephrotic syndrome

Steroid-resistant nephrotic syndrome (SRNS) in children is intractable, and the optimal treatment regimen is not known. We investigated the efficacy of methylprednisolone pulse therapy (MPT) in SRNS patients. Using only MPT plus heparin, we have treated ten pediatric SRNS patients that were resistant to cyclophosphamide (CPM) or cyclosporin A (CsA) and predicted to have a very poor prognosis. Administration of 30 mg/kg per day methylprednisolone with heparin for 3 days was taken as one course, and this was given 14 times over 2 years. One patient discontinued MPT because of peritonitis. Of the remaining nine patients, complete remission was achieved by four patients, persistent mild proteinuria remained in three patients, and no effect was observed in two patients. All patients that had been diagnosed with minimal change (MC) pathology (n =3) at the initial renal biopsy achieved complete remission. Observed adverse events were peritonitis in one patient and a transient decrease in pulse rate only during MPT in one patient. These results demonstrate that MPT with heparin can induce remission in children with SRNS, even when the patient is resistant to CPM and CsA.