Steroid sensitive nephrotic syndrome

The incidence of steroid sensitive nephrotic syndrome (SSNS) is 1.8-16.9 per 100,000 children aged below 15 years. The incidence is much higher in children of South Asian ancestry. The majority of children with SSNS have minimal change disease but some children with mesangial proliferative glomerulonephritis or focal and segmental glomerulosclerosis also respond to corticosteroids. The aetiology and pathogenesis of SSNS remains uncertain although the evidence supports a role for both T and B cells. About 80% of children with SSNS suffer a relapsing course and are at risk of the complications of nephrotic syndrome and of therapy with corticosteroids and/or corticosteroid sparing agents. Initial and subsequent remissions are achieved with high doses of corticosteroids. Children with frequent relapses and/or significant corticosteroid related adverse effects may receive additional treatment with one or more steroid-sparing agents including levamisole, mycophenolate mofetil/mycophenolate sodium, cyclophosphamide, calcineurin inhibitors (cyclosporin, tacrolimus) and rituximab. This chapter provides information on the epidemiology, aetiology and pathogenesis, histopathology, therapies and outcomes of children with SSNS.