minimal change nephropathy ( mcn ) accounts for 25% of adults presenting with a nephrotic syndrome .
although most patients respond to corticosteroid therapy , a significant number relapse frequently and may present a real therapeutic difficulty .
we present a case of apparently refractory relapsing mcn that was successfully treated with a combination of sirolimus and cyclosporin .
in 1989 , a 35-year - old female presented acutely with heavy proteinuria ( 22 g/24 h ) and normal renal function .
she was commenced on oral prednisolone ( 60 mg od ) , and achieved complete remission within weeks . however , multiple relapses followed on reduction of the corticosteroid dose .
azathioprine , cyclosporin , mycophenolate mofetil , tacrolimus and chlorambucil were all tried unsuccessfully , but neither the relapse rate nor the steroid requirements improved . in 2003 , cyclosporin was re - introduced to minimize steroid exposure with trough plasma levels of 149 g / l . in the preceding 14 years , the prednisolone dose had rarely been below 15 mg / day , and there had typically been two to three relapses per year . in 2006 , sirolimus 2 mg / day was added to her prednisolone ( 15 mg / day ) , and cyclosporin ( 6 mg / kg / day)she was also prescribed primidone since childhood for epilepsy . at review , the trough plasma levels of cyclosporin and sirolimus were 153 g / l and 2.9 g / l , respectively .
steroids were gradually reduced and subsequently completely discontinued 24 months after sirolimus was added with no relapses in the last 30 months .
mcn is predominantly a steroid - responsive disease , with around 75% of adult patients achieving remission by 8 weeks .
unfortunately , 3060% will suffer at least one episode of relapse and around 25% will do so more frequently .
a variety of immunosuppressive medications , each with its own side effect profiles , have been trialled as steroid - sparing agents with variable success . frequently relapsing minimal change disease
the first documented use of sirolimus in combination with tacrolimus as a therapeutic option in mcn was published in 2005 by patel et al . .
in conclusion , we have detailed a case of relapsing mcn , which has only been controlled by the combined use of sirolimus and cyclosporin .
this has not only resulted in the longest period of remission , but has also allowed for the discontinuation of corticosteroids .
this is the second documented use of sirolimus in this manner that we are aware of and the first that has trialled the combination of sirolimus and cyclosporin .
 minimal change nephropathy ( mcn ) accounts for around 25% of adults presenting with a nephrotic syndrome . 
 although most patients respond to corticosteroid therapy , a significant number relapse frequently and may present a real therapeutic difficulty . 
 we present a case of apparently refractory relapsing mcn that was successfully treated with a combination of sirolimus and cyclosporin , resulting in the longest period of steroid free remission that the patient has ever experienced . to our knowledge , this is the first documented use of this combination in this manner . 