Abstract
Steroid-resistant nephrotic syndrome is sometimes unresponsive to not only oral prednisolone therapy, but also steroid-pulse therapy or various immunosuppressants. We report a case of steroid-resistant nephrotic syndrome who treated with the combination of intermittent steroid pulse therapy and oral cyclophosphamide successfully although she was resistant to either steroid pulse mono-therapy nor the combination of oral prednisolone and cyclosporine or cyclophosphamide. A three-years-old girl with nephrotic syndrome was refractory to oral prednisolone therapy of two weeks. Subsequent methyl-prednisolone pulse therapy of three weeks reduced her proteinuria but could not introduce into remission. Renal biopsy indicated minor glomerular abnormalities and there was no sclerotic lesion. On the immuno-fluorescence study, no deposition of immunoglobulin and complements was detected. Since her proteinuria increased and edema became severe after the pulse therapy, she required the intravenous supplementation of albumin almost every day. Her proteinuria was resistant also to cyclosporine and subsequent oral cyclophosphamide with oral predonisolone. So we tried the combination of methyl-prednisolone pulse therapy and oral cyclophosphamide. This combined therapy decreased her proteinuria, but did not introduced into the remission. Two weeks after the combined pulse therapy, thrice per week of steroid pulse therapy were performed again alternate weekly three times. During this intermittent steroid pulse therapy, her proteinuria had become negative. After the alternate weekly methyl-prednisolone pulse therapy, she is treated with the pulse therapy once a week at our out-patient clinic. Some authors reported the effectiveness of the combination of methyl-prednisolone pulse therapy and immunosuppressants such as cyclophosphamide or cyclosporine for the patients with focal segmental glomerular sclerosis, although international study of kidney disease in children denied the benefit of cyclophosphamide. In present case, even during steroid pulse therapy of three weeks with cyclophosphamide, proteinuria was reduced but not disappeared, and subsequent intermittent steroid pulse therapy had dissipated her proteinuria. We consider that the combined therapy of intermittent steroid pulse and cyclophosphamide is useful for the treatment of nephrotic syndrome resistant to various immunosupressive therapy.
