Idiopathic focal segmental glomerulosclerosis (FSGS) is a primary glomerular disease that essentially represents a form of chronic, progressive renal fibrosis for which there is no discernible cause. Often presenting with or eventually manifesting the nephrotic syndrome, this disease is increasing in incidence in both children and adults. Therapy continues to be a challenge, although some patients clearly respond to corticosteroids or cyclosporine with a decrease in, or remission of, proteinuria. A favorable response is associated with a decreased likelihood of progression to kidney failure. Given our clinical experience and recent advances in understanding the genetics of FSGS, a stochastic model of disease pathogenesis can be proposed.
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