Long-Term Outcome of Children with Steroid-Sensitive Idiopathic Nephrotic Syndrome

In children, the most frequent glomerular disease is idiopathic nephrotic syndrome, which is defined by the combination of a nephrotic syndrome and nonspecific histologic abnormalities of the kidney including minimal changes, FSGS, and diffuse mesangial proliferation. The term minimal-change disease has become synonymous with steroid-sensitive idiopathic nephrotic syndrome, although renal biopsy is usually not performed for patients who respond to steroid therapy. Indeed, the response to steroid therapy carries a greater prognostic weight than the histologic features seen on initial renal biopsy.Up to 80% of children with idiopathic nephrotic syndrome respond to corticosteroids, with a complete remission occurring within 30 d. Approximately one third of these patients have only one attack and are definitively cured after the course of corticosteroids. Ten to 20% of patients experience relapses several months after stopping the treatment and a cure takes place after three or four episodes, which respond to a standard course of corticosteroids. The remaining 40 to 50% of patients experience frequent relapses either as soon as steroid therapy is stopped (frequent relapsers) or when the dosage of steroids is decreased (steroid dependent). These steroid-dependent patients may have a prolonged course. In the long term, the risk for relapse and the …