Steroid resistance in prolonged type I membranoproliferative glomerulonephritis and accelerated disease remission after steroid withdrawal

Abstract

Two cases of severe proteinuria and hypocomplementemia were referred to our out patient clinic for continuous follow-up. Initial onset of clinical symptoms was at the age of 15 years in both cases. They had already been diagnosed as type I membranoproliferative glomerulonephritis (type I MPGN) by renal biopsy when oral prednisolone administration had been initiated. Several courses of steroid pulse therapy were performed for the flares of the disease, resulting in only temporary amelioration of renal symptoms. Percutaneous renal biopsy was performed during admission on both cases, showing severe glomerular and tubulointerstitial damages, in addition to typical type I MPGN findings such as mesangial cells and matrix interposition and subendothelial deposits. Because the continuous administration of steroid for more than 10 years did not ameliorate the clinical symptoms, steroid was markedly reduced or stopped in these cases. Such withdrawal from steroid therapy accelerated the amelioration of renal symptoms, including decrease in proteinuria, elevation of plasma protein and complement levels, and disappearance of generalized edema. The clinical courses of these cases indicate clinical choice of withdrawal from steroid therapy as one of the treatments in prolonged type I MPGN, which presents in childhood and shows steroid resistance.