focal segmental glomerulosclerosis is the second most common cause of idiopathic nephrotic syndrome ( ns ) in adults .
kidney transplantation in patients with fsgs has a recurrence rate of 2040% after first transplant and up to 5080% in subsequent transplants .
plasmapheresis has been used as mainstay of therapy for recurrence ; however , most patients relapse after cessation of plasmapheresis .
, we report a case of posttransplant recurrent fsgs , who was successfully managed with addition of angiotensin receptor blockers ( arbs ) without plasmapheresis .
a 52-year - old male with end - stage kidney disease ( esrd ) on maintenance hemodialysis since may 2011 presented to us in july 2011 .
his basic disease was unknown . on evaluation , he had bilaterally small kidneys with urine output of only 200300 ml / day .
he underwent kidney transplantation on august 2 , 2011 , with triple immunosuppression consisting of tacrolimus ( tac ) , mycophenolate mofetil , and prednisolone without antibody induction .
initially , he had good urine output and creatinine decreased to 1.6 mg on day 4 ; however , on postoperative day 5 , patient became oliguric .
renal doppler was normal , so a kidney biopsy was done on day 6 , and hemodialysis was initiated .
biopsy revealed changes of acute cellular rejection ( acr , banff ib ) with acute tubular necrosis ( atn ) .
patient received three doses of intravenous methylprednisolone 500 mg , but his urine output and kidney function did not improve and he was continued on dialysis .
a repeat kidney biopsy on day 16 revealed focal atn without inflammation and patient was continued on dialysis .
a third kidney biopsy was done on day 30 in view of nonrecovering renal failure , which showed recovering atn and changes of calcineurin inhibitor toxicity .
tac doses were reduced and subsequently his urine output increased and renal functions gradually improved .
he achieved a minimum creatinine of 1.3 mg / dl at 2 months of transplant . in his next visit after 2 weeks , patient developed swelling over feet .
a 24 h urine collection confirmed proteinuria of 11.8 g / day . at this time , his serum albumin was 1.7 mg / dl , and creatinine , 1.4 mg / dl .
a repeat kidney biopsy was done at 3 months , which revealed segmental sclerosis in 3/22 glomeruli on light microscopy ( lm ) .
there was no interstitial inflammation or tubular atrophy / interstitial fibrosis ( ifta ) in biopsy [ figure 1a ] .
electron microscopy revealed diffuse foot process effacement confirming the diagnosis of recurrent fsgs [ figure 1d ] .
patient was explained about the need of pp ; however , he did not agree for the same .
hence , his prednisolone dose was increased to 30 mg / day and tab telmisartan 40 mg / day was added , which was gradually increased to 120 mg / day .
prednisolone was gradually tapered to 7.5 mg / day over next 6 months . with this treatment , there was a gradual improvement in proteinuria , which decreased to 600 mg / day at the end of 1 year and creatinine remained between 1.2 and 1.4 mg / dl .
serum albumin also increased to 3.2 g. after 1 year , proteinuria again increased to 2 g / day on reducing telmisartan to 40 mg / day , as he had hypotension and dizziness .
a repeat kidney biopsy revealed global sclerosis in 3/19 glomeruli , segmental sclerosis with hyalinosis in 6 glomeruli , synechia formation in 3 glomeruli , and ifta in 1015% of the cortical area [ figure 1b and c ] .
biopsy findings suggested that despite control of proteinuria and apparently normal serum creatinine , the histological changes continued to progress . at this time again , treatment with plasmapheresis and rituximab was discussed with patient , however he was not willing .
subsequently , his prednisolone was increased to 20 mg / day and telmisartan to 120 mg / day .
this resulted in gradual improvement in proteinuria to 500 mg / day over the next 1 year .
now , after completion of 4 years of transplant , patient is doing well with serum creatinine of 1.41.5 mg / dl , and proteinuria between 250 and 500 mg / day .
his current immunosuppression includes tab prednisolone 7.5 mg / day , mycophenolate sodium 360 mg twice daily , and tac 1 mg twice daily ( level 45 ng / ml ) .
( a ) by light microscopy , the initial biopsy show focal segmental glomerulosclerosis ; however , no tubular atrophy or interstitial fibrosis was noted ( b ) by light microscopy , the following biopsy show focal segmental glomerulosclerosis , occasional globally sclerosed glomerulus along with mild patchy tubular atrophy occupying 1015% of the sampled cortex ( c ) higher magnification of the 5 allograft biopsy show lesion of segmental sclerosis with hyalinosis and synechiae formation ( d ) electron microscopy performed on 4 allograft biopsy show extensive foot process effacement
the risk factors for recurrence are early age of presentation of ns , rapid progression to esrd , presence of mesangioproliferation in kidney biopsy , and retransplant , etc .
early recurrence is more common , occurs within few days of transplant and is associated with massive proteinuria and delay in treatment may lead to rapid deterioration of renal function .
kidney biopsy within few days after transplant is normal on lm , except the presence of diffuse foot process effacement in electron microscopy .
another feature of recurrent fsgs found in some reports is higher chances of acute rejection and atn .
our patient had delayed graft function and initial biopsies within 1 month revealed changes of acr and atn without any segmental sclerosis . however , as kidney function improved , patient was found to have heavy proteinuria with hypoalbuminemia and kidney biopsy at 3 months confirmed recurrence of fsgs [ figure 1a and d ] .
our patient did not have pretransplant biopsy , so there was a possibility of de novo fsgs ; however , de novo fsgs usually presents late .
one series found that the mean time of diagnosis of de novo fsgs after transplantation was 57 months .
most studies demonstrate remission rates between 50% and 60% with pp , but most patients relapse after stopping pp , suggesting that such treatment induces transient rather than sustained remission and graft survival is significantly inferior to patients without recurrence .
it is possible that , despite initial remission of proteinuria , the histological changes continue to progress .
our patient had progression of histological changes at 1 year , despite control of proteinuria and normal renal function .
some reports have advocated intensifying immunosuppression , for example , replacement of azathioprine with cyclophosphamide or using high doses of cyclosporine .
angiotensin - converting enzyme inhibitors ( aceis ) are shown to reduce proteinuria in one case report , but in this report aceis were used with pp .
our patient responded to the slight increase in dose of corticosteroids , and use of arb without pp and is doing well after 4 years of transplant .
savin et al . demonstrated that a circulating factor of 3050 ka protein is associated with increased permeability to albumin in rats .
wei et al . demonstrated that soluble urokinase - type plasminogen activator receptor ( supar ) is most likely this factor ; however , more recent work has shown that supar is not correlated with proteinuria in recurrent fsgs and increased levels are found in other causes of proteinuria as well as other diseases . to summarize , this case report highlights two things : first and foremost is that some patients with post - transplant fsgs might respond to mild increase in steroid doses and optimal use of ace inhibitors / arb without pp and second despite the clinical response , the histological lesions may continue to progress .
 recurrence of focal segmental glomerulosclerosis ( fsgs ) is common after kidney transplantation . 
 plasmapheresis ( pp ) is considered to be the most effective treatment ; however , results are variable and relapse is common after stopping plasmapheresis . here , we report an unusual case of recurrent fsgs , who achieved complete remission with angiotensin receptor blocker therapy . 
Introduction
Case Report
Discussion
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