The volume of plasma to achieve remission in atypical hemolytic uremic syndrome

Introduction: Poor prognosis is anticipated in children with FSGS despite various treatment options. Herein we report a case with good prognosis although very expensive and still challenging regarding the unknown long term side effects of Rituximab.Case report A 34-month old girl presented with features of nephrotic syndrome on 22/02/2010. She was resistant to a classic course of 60mg/m2 daily prednisolone. A kidney biopsy revealed tip variant FSGS with 5%interstitialfibrosis.Two doses of Rituximab, 375/m2, was prescribedas well as tacrolimus 0.5mg q12h which was added to her low dose alternate day prednisolone. It took about a year for her to respond completely. Six months later she had a relapse which responded to increasing the dose of tacrolimus within 3 months, another relapse occurred 4 months later with a tacrolimus level of 7.8ng/ml; therefore, 2 more doses of Rituximab were prescribed on 25/09/2012. Since this date until her last relapse on 25/10/2017 sheexperienced 6 more relapses, with 10-13kg weight gain and a response to 2 doses of Rituximab within 2months on each relapse. Meanwhile she was on enalapril, spironolactone,low dose prednisolone and tacrolimus. Until now she has received 16 doses of rituximab. Conclusion:This was a challenging case of rituximab dependent nephrotic syndrome(if we can use this term for the first time) and even more challenging is the frequent doses of rituximab with unknown untoward long term side effects.

کلیدواژه ها:

nephrotic syndrome ، FSGS ، rituximab dependent