Focal segmental glomerulosclerosis with idiopathic nephrotic syndrome: three types of clinical response

Abstract

In a retrospective analysis, 51 patients with focal segmental glomerulosclerosis and idiopathic nephrotic syndrome, who were treated with steroid or cyclophosphamide therapy, were divided into three clinical groups according to the remission profile of their nephrotic syndrome. Group 1 patients (19.37%) consistently responded to medication; none has progressive renal failure (mean follow-up 10.6 years). Group 2 patients (25, 40%) failed to respond to medication; terminal renal failure has occurred in 12 of them. Group 3 patients (7, 14%) initially appeared to be responsive to medication and continued to respond for up to 18 months, but subsequently became unresponsive to any therapy; five of them have required dialysis or transplantation. This third group of patients could not be separated clinically or pathologically from group 1 patients, all of whom have an excellent prognosis. One should, therefore, be cautious about predicting the outcome of steroid-responsive nephrotic patients, especially those with FSGS, until at least 18 months after the onset of illness.