The importance of focal segmental glomerulosclerosis (FSGS) has been gradually increasing in childhood steroid resistant nephrotic syndrome (SRNS). Although the exact pathogenesis of the FSGS is not known, underlying mechanisms of FSGS fall into four categories; humoral factors, hemodynamic factors, podocyte injury, and genetic predisposition. There exist some challenges of treatment with FSGS in childhood. Steroids and cytotoxic agents are generally ineffective in inducing remission in FSGS. Treatment with cyclosporine might be succesfull in reducing proteinuria. However, complete remission rate with cyclosporine is quite low in FSGS on the long-term. Even fewer data are available on success rate of the use of new therapeutic agents such as tacrolimus and mycophenolate mofetil in FSGS. The assesment of the role of plasmapheresis in the management of patients with FSGS requires further evaluation. Non-immunosuppressive therapy (i.e. angiotensin-converting enzyme inhibitors, lipidlowering drugs, non-steroidal antiinflammatory drugs) should be applied to almost all patients with FSGS. Because there is no treatment with utmost effectiveness and the prognosis is poor with progression to chronic renal failure, FSGS represents a significant therapeutic dilemma for pediatric nephrologists. In this review, the pathogenesis and therapeutic options on FSGS were described.

Keywords: Steroid Resistant Nephrotic Syndrome, Focal Segmental Glomerulosclerosis