Alternatives to steroid treatment of steroid-sensitive nephrotic syndrome in childhood

Background. Despite being an orphan disease, idiopathic nephrotic syndrome in childhood is the most frequent glomerular disease in this age group. Nephrotic syndrome in children is a heterogeneous disease and in order to assess the individual facets of the disease a classification using the following criteria are helpful: etiology, age at onset, histology and responsiveness to initial standard treatment with glucocorticoids. Most important is the differentiation between steroid-sensitive (SSNS) and steroid-resistant nephrotic syndromes (SRNS) because SRNS is a risk factor for developing end-stage renal disease. Conclusion. A high cumulative dosage of glucocorticoids and prolonged intake do not seem to impact on the risk of relapse. In order to avoid short and long-term glucocorticoid-associated side effects alternative treatment options should be kept in mind when planning individual treatment options, especially with mycophenolate mofetil, calcineurin inhibitors and rituximab in patients with frequently relapsing disease. Genetic diagnostics aremandatory in patients with SRNS; however, immunosuppressive treatment in children with genetic forms of nephrotic syndrome rarely leads to remission.