Abstract

Background: Steroid resistant nephrotic syndrome due to Idiopathic Focal Segmental Glomerulosclerosis (FSGS) or genetic mutations, is one of the most common causes of End Stage Renal Disease (ESRD) that leads to renal transplantation. Relapse of the disease in transplanted kidney despite all proper therapeutic managements prior and post transplantation may lead to graft loss. Lack of precise data about the status of transplanted patients due to FSGS, makes us to obtain data from all pediatric nephrologists in Iran to get better pre and post transplantation therapeutic managements. Material and methods: All data obtained via questionnaire that contains information about our colleagues’ personal data, surgical and medical management prior to transplantation, relapse of disease in allograft kidney and therapeutic response rate after relapse. Results: Of 82 cases of FSGS since 1998 to 2018, 23 cases had relapse, most of them in less than 1 year after transplantation. When relapse occurred, nearly all centers used plasmaphresis and rituximab and some used Angiotensin Converting Enzyme Inhibitor (ACEI) or Angiotensin Receptor Blocker (ARB) in addition to immunosuppressant medications and methyl prednisolone pulse therapy. Genetic studies were done only in two centers and there was no difference in immunosuppressant medications between these two centers and idiopathic group. Pre-transplant plasmaphresis and rituximab were done in four centers, while two centers used IVIG and one center only used plasmaphresis. Delayed graft function (DGF) was negative in 7 and positive in 9 centers. In most centers immunosuppressant medications consisted of corticosteroid, mycophenolate mofetile and tacrolimus. Relapse and recovery rates were variable from less than 10% to more than 50% in all centers. Seven centers had no response to any medications. Lowest relapse rates were in 2 centers that had deceased donors and used rituximab and plasmaphresis prior to transplantation. Conclusion: Therefore, it is concluded that with regard to possibility of relapse after transplantation and variable therapeutic management before and after transplantation, it is reasonable that genetic analysis of mutation and determination of idiopathic and high risk cases and using appropriate therapeutic protocols, should be done to decrease relapse rate.