Treatment of steroid-resistant nephrotic syndrome

Steroid-resistant nephrotic syndrome (SRNS) is associated with different histological types of glomerulopathies (GN), which - although showing the same picture on histology - may have different aetiologies. The defective podocyte is at the centre of pathogenesis of all SRNS; thus, SRNS may be referred to as podocytopathies. This review summarises the state of the art of treatment of SRNS in 2008. The vast majority of published manuscripts have ignored the aetiological and histological heterogeneity of SRNS. Most authors nonselectively attributed their patients of all age groups to idiopathic types of SRNS. This led to the considerable risk of misinterpreting successful or unsuccessful treatment schedules. Sadly, there is a lack of randomised, controlled multicentre trials on SRNS in children. In our own paediatric nephrology unit, with more than 200 children with SRNS, one-quarter of all patients had genetic types of GN, one-quarter had secondary types, and half had idiopathic GN. Almost no patients with the genetic form of SRNS responded to immunosuppression. The effect of treatment of secondary types of SRNS depended on the efficacy of treatment of the underlying disease. In patients with idiopathic types of SRNS, the percentage of complete remissions after induction therapy ranged between 50% and 88%, according to the pathohistological type of GN.