Management of steroid resistant nephrotic syndrome: An experience with cyclosporine-A

Objective: to evaluate the effectiveness and safety of cyclosporine-a as the mainstay of treatment for induction of complete and sustained remission in steroid resistant nephrotic syndrome in children. design: a hospital based descriptive study (case series). place and duration: renal clinic of department of pediatrics combined military hospital lahore,pakistan. from december 1999 to december 2003. patients & methods: twenty nine patients of steroid resistant nephrotic syndrome (focal segmental glomerulosclerosis in 13 cases,minimal change disease in 8,mesangioproliferative glomerulonephritis in 4,diffuse membranoproliferative glomerulonephritis in 3 and crescentic glomerulonephritis in 1) with normal renal function and glomerular filtration rate,were treated with cyclosporine-a and enalapril for 12 months along with low dose alternate day prednisolone tapered over six to nine months. results: mean age of the 29 patients (20 boys and 9 girls) was 8.3 years (range: 2.7-14). mean age at the onset of nephrotic syndrome and commencement of cyclosporine-a trial was 4.3 years (range 1.7-12) and 8 years (range: 2.5-14) respectively. 'early steroid resistance' was observed in 7 (24.1%) cases and 'delayed steroid resistance' in 22 (75.8%) cases. at the completion of 12 months of treatment,17 (58.6%) patients were in a complete and sustained remission having predominance of focal segmental glomerulosclerosis (9 out 13 cases) and minimal change disease (6 out of 8 cases). eight (27.5%) patients persisted with nephrotic range proteinuria. this group included 6 cases who initially achieved only a partial remission and 2 cases that later turned cyclosporine-a unresponsive after staying in complete remission for some time. four (13.7%) cases never went into remission right from the very beginning. the mean duration to achieve 'complete' and 'partial remission' after the commencement of cyclosporine-a trial was 2.2 months (range 1- 3.5) and 3 months (range 2-5) respectively. the correlation of an early remission with a longer duration of complete and sustained remission was significant. recurrent pyuria was found in 4 (13.7%) and culture proven bacterial peritonitis in 2 cases. hirsutism was reported in 22 (75.8%) patients. three (10.3%) patients progressed to end stage renal disease. conclusion: cyclosporine-a is reasonably effective and safe in inducing complete and at least 12 months long sustained remission in about 60% cases of steroid resistant nephrotic syndrome in children,particularly when it leads to an early remission.