a 19-year - old female with no significant past medical history or medication use presented to our hospital in april 2010 with severe nephrotic syndrome characterized by marked fluid retention , heavy proteinuria ( protein : creatinine ratio of 1244 mg / mmol ) , hypoalbuminaemia ( 13 g / l ) and elevated total serum cholesterol ( 9.3 mmol / l ) . renal function was preserved with a serum creatinine of 42 mol / l , and there was no haematuria evident on urine microscopy . lupus and vasculitis markers , hepatitis serology and serum electrophoresis were negative . a renal biopsy was performed which revealed normal glomeruli , tubules and interstitium . the patient was commenced on prednisolone 50 mg daily ( 1 mg / kg ) in addition to ramipril 5 mg daily , simvastatin 10 mg daily and warfarin therapy . complete remission was achieved within 2 months and prednisolone dose was reduced to 25 mg daily . soon after , and against medical advice , the patient abruptly ceased her prednisolone and within 72 h had a relapse of disease with recurrence of gross peripheral oedema , hypoalbuminaemia of 13 g / l ) and proteinuria of 1030 mg / mmol ( from 0.06 mg / mmol ) . despite recommencing high - dose steroids ( 60 mg / day ) , the disease thereafter remained refractory to steroid therapy with persistent gross oedema , nephrotic - range proteinuria and hypoalbuminaemia ( albumin of 9 g / l ) . after failing to respond to 3 months of high - dose steroids , the patient was commenced on cyclophosphamide . in light of the patient 's history of non - compliance and in an effort to reduce drug exposure and toxicity , pulse intravenous cyclophosphamide was administered in incremental doses of 0.5 , 0.75 and 1.0 g / m 1 month apart . during this period two months after the third pulse of cyclophosphamide was administered , there was no evidence of disease response with ongoing gross oedema , hypoalbuminaemia ( albumin 14 g / l ) and heavy proteinuria ( 1476 mg / mmol ) . at this point , it was decided to trial the monoclonal anti - cd20 antibody rituximab ( rtx ) , with two doses of 500 mg given 2 weeks apart . complete remission was rapidly induced within 1 month of rtx therapy with resolution of oedema , rising albumin to 31 g / l and absent proteinuria ( figure  1 ) . 1.changes in serum albumin ( g / l ) and urine protein : creatinine ratio ( mg / mmol ) over time , relative to treatment modality  . changes in serum albumin ( g / l ) and urine protein : creatinine ratio ( mg / mmol ) over time , relative to treatment modality  . the patient has remained in complete remission for more than 32 months since the two doses of rtx were administered in february 2011 . this is despite recovery of cd19 counts back to normal levels 24 months after treatment . mcd is the most common cause of nephrotic syndrome in children and accounts for 1025% of cases in adults . close to 75% of cases will respond initially to a course of high - dose glucocorticosteroids but relapse commonly occurs . steroid - sparing agents like calcineurin inhibitors and cyclophosphamide are often used for frequently relapsing or steroid - dependent mcd . besides the significant adverse side effects of these agents , a large proportion of patients relapse after cessation of these medications . while there was some suggestion that oral cyclophosphamide may be more successful than iv cyclophosphamide in achieving disease remission in refractory mcd , larger clinical trials have suggested that both forms of therapy are equivocal with respect to relapse rates and toxicity . there are an increasing number of reports of success of rtx therapy for steroid- and cyclosporine ( csa)-dependent nephrotic syndrome in paediatric populations [ 4 , 5 ] . a single rtx infusion also appeared to improve response to csa therapy and had significant steroid - sparing effects in children with steroid-dependent nephrotic syndrome ( sdns ) who had developed secondary resistance to csa . in a long - term follow - up study of 37 children who received rtx for sdns , 26 patients remained in remission after 12 months and 7 remained in remission after 24 months without further maintenance immunosuppression . however , response to rtx has been mixed in cases of steroid-resistant nephrotic syndrome ( srns ) in children . reported the successful use of rituximab in five patients with srns , two of which had mcd . .   carried out a multi - centre questionnaire - based study in which the response rate in children with srns was as high as 44% . in another case series of four children with srns , including one case of mcd , all children failed to achieve sustained remission after a single dose of rituximab , despite complete b - cell depletion . in adults , the first case report of the efficacy of rtx in multi - relapsing mcd was published in 2007 . since then there have been a number of reports of success with rituximab in steroid - dependent mcd [ 12 , 13 ] . munyentwali et al .   analysed the outcome of 17 adult patients who were treated with rtx for steroid - dependent mcd over a mean follow - up period of 29.5 months . after the first course of rtx , 11 patients ( 65% ) did not relapse after a mean follow - up of 26.7 months . twelve of the 17 patients ( 70% ) were free of any immunosuppressant drugs at last follow - up . there are minimal data available regarding the use of rtx in steroid - resistant mcd in adults . the first case report , published in 2008 , describes an adult patient with steroid- and mycophenolate mofetil - resistant mcd who remained in complete remission 1 year after initiation of rtx therapy . a further case report was of a 23-year - old patient with steroid- and csa - resistant mcd treated with two doses of rxt with sustained complete remission at the end of 1 year . the second dose of rtx was given at 6 months in response to a recovery of b - cell counts . a long - term follow - up study was published recently by bruchfeld et al .   on 16 adult patients with biopsy - proven mcd of which 13 were steroid dependent , 2 multi - relapsing and 1 steroid and multi - drug resistant . in this study , the rtx dose varied from 1000 to 2800 mg . complete remission was achieved after initial rtx treatment in 13 patients . in the remaining cases , partial remission was achieved in all except the patient with steroid- and multi - drug - resistant disease . relapses can often occur after the reappearance of cd19 cells , although this is not always the case [ 12 , 14 ] . guigonis et al .   found a consistent association between relapses ( 3 of 22 paediatric patients ) and an increase in cd19 cells which occurred at a median time of 11 months ( range 839 months ) . sellier - leclerc et al .   evaluated disease outcome after a minimum cd19 depletion period of 15 months obtained by repeated rtx infusion and found that it induced long - term remission even after definitive cd19 recovery in almost two thirds of the paediatric patients without the use of maintenance oral immunosuppressive drugs . the rtx dosing has varied greatly in various reports including a single dose , doses of 500 or 1000 mg given at one or two time - points and 375 mg / m once weekly for 4 weeks . in the study by munyentwali et al . we report a unique case of steroid- and cyclophosphamide - resistant mcd responding rapidly to two doses of rtx at 500 mg each , with sustained complete remission even after cd19 recovery . as discussed , rtx has been shown to be efficacious in a growing number of small case series in steroid - sensitive and steroid - dependent mcd ; however , reported success of its use in multi - drug - resistant disease , particularly in adults , remains limited . in our case , clearly , a well - conducted multi - centred clinical trial of rtx for mcd is warranted either as a first - line agent or in cases of steroid - dependent and/or -resistant disease . the results presented in this paper have not been published previously in whole or part , except in abstract format .