Therapies for steroid-resistant nephrotic syndrome

Abstract

Between 10 and 20% of children with primary nephrotic syndrome are steroid-resistant (SRNS). From earlier studies in children with SRNS, we know that cyclosporin (with or without alternate-day prednisone) and cyclophosphamide (with pulse intravenous corticosteroids) result in comparable complete or partial remission rates of about 60%. An evaluation of the relative effectiveness of cyclophosphamide and cyclosporin has not been possible because of the absence of a head-to-head randomised trial. The Arbeitsgemeinschaft für Pädiatrische Nephrologie trial, published in this issue of Pediatric Nephrology, has filled this gap in our evidence base. Although there was no difference in the number of patients achieving complete remission, those patients receiving cyclosporin treatment were significantly more likely to achieve partial remission than those receiving intravenous cyclophosphamide. This result suggests that cyclosporin rather than cyclophosphamide should be used as first line therapy for children with SRNS.

Collapsing Focal Segmental Glomerulosclerosis in a Young Child

“…Our results, which we have published, demonstrate that in our hands we are able to achieve a 60% remission rate with CsA in SRNS [34], which is similar to other published data [35]. I should add that if the kidney function is impaired, we use mycophenolate mofetil instead of CsA due to concerns regarding the nephrotoxicity of CsA.…”

Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study

“…There is a considerable diversity in the use of immunosuppressive drugs, with differences in combinations, administration modes and dosage regimens. Optimal strategies with least toxicity remain to be determined [12,13].…”

Comparison of Direct Action of Thiazolidinediones and Glucocorticoids on Renal Podocytes: Protection from Injury and Molecular Effects

“…Unfortunately, GCs have serious side effects, and in ϳ20% of patients, they are ineffective in inducing clinical remission of disease (i.e., steroid-resistant NS). Thus, it is clear that alternative therapies with greater efficacy and/or less severe side effects are critically needed (Hodson and Craig, 2008).…”