Treatment of focal segmental glomerulosclerosis

Focal segmental glomerulosclerosis (FSGS) has been increasing in incidence over the past 2 decades and may currently be the most common form of primary nephrotic syndrome in the United States. Nephrotic patients with FSGS who do not achieve a remission in proteinuria usually advance to end-stage renal disease within 5 to 10 years. Although initially felt to be a steroid-resistant disease, especially in adults, studies show significant responsiveness to more prolonged courses of steroids. For patients with steroid-resistant or steroid-dependent FSGS, cyclosporine A and cytotoxic agents have shown efficacy in clinical trials. Other agents used include pulse methylprednisolone, azathioprine, tacrolimus, mycophenolate mofetil, and combination therapy. For recurrent FSGS after renal transplantation, plasmapheresis is often used but appears not to be as efficacious in adults as in the pediatric population.     

Treatment of focal segmental glomerular sclerosis with rituximab: 2 case reports

BACKGROUND: Primary focal segmental glomerular sclerosis (FSGS) recurs in 20 - 40% of patients after kidney transplantation. Rituximab has been used to treat several glomerular diseases. PATIENTS AND RESULTS: We treated two renal-transplant patients with recurrence of FSGS with rituximab. Despite a prophylactic perioperative therapy of plasmapheresis (PE) and i.v. cyclosporine A, Patient 1 developed significant proteinuria, at 1 day after his first kidney transplantation. After two infusions of rituximab (375 mg/m2) he had complete remission. A second relapse, which occurred on Day 40, was also successfully treated by PE and one additional infusion of rituximab. 10 months after transplantation, he still has complete remission from recurrent nephrotic syndrome. Patient 2 also developed significant proteinuria, but 1 day after a second kidney transplantation. Nephrotic syndrome persisted despite 27 sessions of PE and cyclophosphamide therapy. At 13 months after transplantation, he received four infusions of rituximab (375 mg/m(2)), but this was ineffective. CONCLUSION: There is a need to demonstrate whether or not rituximab therapy is of interest to prevent and to treat nephritic syndrome in renal-transplant patients who suffer from FSGS.     

Recurrent nephrotic syndrome after living-related renal transplantation resistant to plasma exchange: report of two cases

Abstract:  We encountered two patients of recurrent nephrotic syndrome (NS) after renal transplantation that was resistant to plasma exchange (PEX). Case 1 was a 34-year-old man with a living-related renal transplant for type-I membranoproliferative glomerulonephritis (MPGN) related end-stage renal disease (ESRD). He developed overt proteinuria 7 months post-transplant and presented with NS 5 months later. Biopsy of the transplant kidney revealed recurrent type I MPGN, but no features of acute rejection (AR) or chronic allograft nephropathy (CAN). He was treated with cyclophosphamide (CP), oral prednisolone (40 mg/d), an anti-platelet agent, heparin sulfate, and PEX, but the nephrotic state persisted and renal function was deteriorated. He recommenced hemodialysis 3 yr and 9 months after renal transplant. Case 2 was a 47-year-old male who underwent living-related renal transplant for ESRD due to focal segmental glomerulosclerosis (FSGS). He presented with proteinuria shortly after renal transplantation. He also had frequent episodes of AR. Graft biopsy revealed recurrent FSGS. Treatment of pulse methylprednisolone and PEX was transiently effective, but NS relapsed shortly after PEX. Graft biopsy at our hospital showed features of CAN with moderate interstitial fibrosis and tubular atrophy, presence of intraglomerular foam cells but no segmental sclerosis. Treatment with 12 courses of low-density lipoprotein apheresis (LDL-A) reduced proteinuria from 9.6 to 2.0 g/d, and incomplete remission has been maintained for more than 1 yr after LDL-A with slowly progressive renal dysfunction. Despite recent therapeutic advances, including the use of immunosuppressants and PEX, treatment of recurrent disease remains difficult. The LDL-A might be useful in cases with recurrent FSGS resistant to PEX.     

Recurrent minimal change disease post-allograft renal transplant

A young adult was diagnosed with steroid-resistant minimal change disease (MCD) without evidence of focal segmental glomerulosclerosis (FSGS) despite serial and detailed sectioning and screening of the renal biopsy. He received initial treatment with steroids and then cyclosporine plus low-dose steroids for 2 years. Renal function progressively deteriorated due to resistance to steroid and cyclosporine. Two months after initiation of hemodialysis, the patient received a living-related allogenic kidney transplant. However, recurrent nephrotic syndrome and renal insufficiency occurred after transplantation. In spite of aggressive treatment, renal function showed no significant improvement. The kidney graft was removed 2 weeks after transplantation. Serial sectioning and thorough sampling and screening revealed no evidence of FSGS, but light microscopy and electron microscopy showed the typical morphologic pattern of MCD, corresponding to the pretransplant diagnosis. We believe that this is the first reported case of recurrent MCD after renal transplant.     

Effect of a triple blockade of the renin-angiotensin-system in recurrent focal segmental glomerulosclerosis after kidney transplantation

Recurrent focal segmental glomerulosclerosis (FSGS) after renal transplantation with nephrotic syndrome is a serious problem with a high risk of graft loss. The therapeutic role of renin-angiotensin-system (RAS) blockers in recurrent FSGS is not clear. We present the safety and efficacy of an intensified triple RAS blockade with an ACE-inhibitor, an AT 1 receptor blocker and the direct renin inhibitor aliskiren in a 29-year-old renal transplant recipient with biopsy proven recurrence of FSGS and relapsing severe nephrotic syndrome. We subsequently used full dose ramipril, candesartan and aliskiren under a close monitoring of kidney function and electrolytes and examined the effect on proteinuria, clinical course and tolerability over 12 months. We found a significant and sustained antiproteinuric effect under triple RAS blockade. RAS blockade was generally well tolerated. This can offer a new therapeutic approach in selected hypertensive patients with recurrent FSGS.     

Decreased proteinuria following liver transplantation in a patient with type C liver cirrhosis complicated with nephrotic syndrome: a case report

Type C liver cirrhosis is often associated with a nephrotic syndrome secondary to membranoproliferative glomerulonephritis. Liver transplantation in such patients may sometimes worsen viremia, causing renal dysfunction upon the introduction of immunosuppressive drugs. We present a case of a patient whose proteinuria decreased after liver transplantation. The patient was a 49-year-old male who had been followed due to chronic hepatitis type C from 1984. From 1999 he was diagnosed as having nephrotic syndrome. We performed a living related liver transplant on August 21, 2001. An intraoperative renal biopsy revealed the histology to show membranoproliferative glomerulonephritis. The volume of proteinuria was 2 to 11 g/day before surgery. After surgery it varied from 6 to 10 g/day, gradually decreasing to 1 to 2 g/day. One of the causes of reduced proteinuria may be alleviation of membranoproliferative glomerulonephritis by immunosuppression. But from the view that the recovery of the renal function followed the recovery of liver function, the major effect may have been alleviated hepatorenal syndrome.     

Potential Donor–Recipient MYH9 Genotype Interactions in Posttransplant Nephrotic Syndrome After Pediatric Kidney Transplantation

Recurrence of focal segmental glomerulosclerosis (FSGS) with nephrotic syndrome is relatively common after kidney transplantation in young recipients whose predialysis course consists of heavy proteinuria, hypertension and subacute loss of kidney function. The gene(s) mediating this effect remain unknown. We report an unusual circumstance where kidneys recovered from a deceased African American male donor with MYH9 -related occult FSGS (risk variants in seven of eight MYH9 E1 haplotype single nucleotide polymorphisms) were transplanted into an African American male child with risk variants in four MYH9 E1 risk variants and a European American female teenager with two MYH9 E1 risk variants. Fulminant nephrotic syndrome rapidly developed in the African American recipient, whereas the European American had an uneventful posttransplant course. The kidney donor lacked significant proteinuria at the time of organ procurement. This scenario suggests that donor–recipient interactions in MYH9 , as well as other gene–gene and gene–environment interactions, may lead to recurrent nephrotic syndrome after renal transplantation. The impact of transplanting kidneys from donors with multiple MYH9 risk alleles into recipients with similar genetic background at high risk for recurrent kidney disease needs to be determined.     

Early recurrent nephrotic syndrome after renal transplantation in children with focal segmental glomerulosclerosis.

BACKGROUND: We analysed risk factors to predict the recurrence of nephrotic syndrome and the therapeutic efficacy of plasmapheresis combined with oral cyclophosphamide (PE+CPM) in early recurrent nephrotic syndrome after transplantation in children with focal segmental glomerulosclerosis (FSGS). METHODS: Medical records after 1990 of 16 children with biopsy-proven idiopathic FSGS and renal transplantation before the age of 18 years were reviewed. RESULTS: Early recurrence of nephrotic syndrome developed in six cases (37. 5%). While early kidney graft biopsies, performed within the first week after the onset of recurrence, revealed diffuse effacement of foot process only, late biopsies contained segmentally sclerosed glomeruli as well. Among several possible risk factors, the mean duration from onset of original nephrotic syndrome to development of end-stage renal disease was shorter in the recurrent group (P=0.045) and the percentage of globally sclerosed glomeruli was higher in the non-recurrent group (P=0.001). PE+CPM therapy resulted in complete remission of nephrotic syndrome if it was started early and if there was no evidence of accompanying acute rejection. CONCLUSION: These results support more liberal use of living-related donors for renal transplantation of children with FSGS and ESRD, considering the shortage of cadaveric donors in our society and relatively good efficacy of the early and intensive PE+CPM therapy for early recurrent nephrotic syndrome.     

Recurrence of nephrotic proteinuria in children with focal segmental glomerulosclerosis after renal transplantation treated with plasmapheresis and immunoadsorption: case reports

Idiopathic focal segmental glomerulosclerosis (FSGS) is believed to be caused by a circulating permeability factor. FSGS recurrence is common after transplantation. The treatment is still a matter of debate; plasmapheresis (PE) and immunoadsorption (IA) are often used. We report on PE and IA in the treatment of two children with recurrent nephrotic proteinuria. Patient 1 was a 16-year-old girl who had recurrence of nephrotic proteinuria on the first day after transplantation (proteinuria-19 g/d). Primary immunosuppressive therapy was changed to high-dose cyclosporine and cyclophosphamide; plasmapheresis was started on day 4. Altogether we performed 53 PE and 38 IA procedures. During the first month, PE procedures were performed with no more than a 2-day interval between sessions, and the girl achieved partial remission (proteinuria 3 g/d). PE was then stopped. After 2 months, a relapse of heavy proteinuria occurred. This relapse was successfully treated again with intensified PE treatment. After achieving remission, a chronic PE regimen was started (PE once a week), similar to the previous series. The child remained in partial remission. Seven months after renal transplantation, she was switched from PE to IA, because of severe hypoproteinemia. Graft biopsy performed at 4 months showed effacement of the foot processes. At the present time she has a good graft function and 3 g/d proteinuria. Patient 2 was a 13-year-old girl with FSGS since 9 years. On the second day after renal transplantation she developed nephrotic proteinuria (proteinuria-14 g/d), which was treated with 39 PE and 16 IA treatments. She went into complete remission on the intensified PE regimen, had one relapse, and was switched to chronic IA. Graft biopsy performed at 2 weeks after transplantation showed effacement of the foot processes. At the present time she has good graft function and low proteinuria (0.3 g/d). In conclusion, intensified PE or IA treatments induced remission of recurrent nephrotic range proteinuria. Chronic PE or IA can maintain patients with frequent relapses in long-term remission.     

Spontaneous remission of post-transplant recurrent focal and segmental glomerulosclerosis

A 12-year-old girl with a history of steroid and cyclosporine (CsA) resistant nephrotic syndrome owing to focal and segmental glomerulosclerosis (FSGS) has progressed to end-stage renal disease (ESRD) for which she underwent hemodialysis for 18 months before she successfully received a fully matched kidney transplant from her sister at the age of nine years. The post transplantation (Tx) period was marked by an early and massive proteinuria indicating recurrent FSGS for which she received 12 sessions of plasmapheresis (PP); unfortunately, she did not appear to have any response to the PP therapy; thereafter, a conservative management comprising essentially enalapril and losartan has been initiated and was also not successful during the first four months, however, a very gradual response has been noticed to occur after five months of conservative therapy and ultimately, the patient attained complete remission after 21 months of treatment. Amazingly, 15 months after discontinuation of enalapril and losartan, she remained in a complete and sustained remission with a good renal allograft function. To the best of our knowledge, this is the first case ever reported in the literature of a "spontaneous" remission of post transplant recurrent FSGS.     

Recurrence of nephrotic syndrome after renal transplantation: influence of increased immunosuppression

Recurrence of nephrotic syndrome after renal transplantation leads to graft loss within 1 year in 50–80% of patients who do not receive any specific treatment. Several treatment protocols have been proposed leading to long-term remission in 50–80% of patients. The aim of our study was to evaluate the efficiency of intensified immunosuppression, simultaneously including methylprednisolone pulses, cyclophosphamide, high-dose cyclosporine and plasma exchanges. Fourteen patients with early recurrence were treated with a protracted high-dose prednisone or IV methylprednisolone, oral cyclophosphamide, high-dose oral or IV cyclosporine, and plasma exchanges. By the end of cyclophosphamide therapy and plasma-exchange program, six out of 14 patients had no proteinuria; five had residual proteinuria without nephrotic syndrome and three experienced ongoing gross proteinuria with nephrotic syndrome. By the end of follow-up, four out of the 14 patients had lost their graft: one out of six with complete remission, one out of five with residual proteinuria and two out of three with persistent nephrotic syndrome. We conclude that multiple reinforcement of immunosuppression in patients with recurrent nephrotic syndrome following renal transplantation as performed in our patients is not more efficient than the single use of cyclophosphamide or plasma exchange or high-dose cyclosporine as reported in the literature.     

Successful treatment of recurrent focal segmental glomerulosclerosis after kidney transplantation by plasmapheresis and rituximab

A 22-year-old patient whose primary kidney disease was focal segmental glomerulosclerosis (FSGS) developed severe recurrence of proteinuria (up to 57 g/24 h) immediately after a haploidentic living donor kidney transplantation despite pre-operative plasmapheresis. The immunosuppressive treatment consisted of tacrolimus, mycophenolate mofetil, basiliximab and steroids. He underwent 10 plasmapheresis sessions in the first 3-week post-transplantation. In addition, he received 2 i.v. doses of rituximab (RTX) 600 mg (375 mg/m(2)) on days 7 and 15. Proteinuria decreased below nephrotic range at day 14 and serum creatinine returned progressively to normal values. A short course of oral ciclophosphamide (100 mg/j) was administrated between days 22 and 40 and three additional plasmapheresis sessions on days 34, 39 and 49. This strategy allowed obtaining sustained full remission of the nephrotic syndrome (NS) and excellent graft function, which persists over 2 years after transplantation. No notable adverse events related to RTX or plasmapheresis were observed. This case suggests that RTX associated with plasmapheresis may be an effective treatment of recurrent NS because of FSGS.     

Recurrent nephrotic syndrome in renal allografts

We describe the clinicopathologic course of two patients with recurrent focal segmental glomerular sclerosis (FSGS). In both patients, FSGS was initially demonstrated during the evaluation of proteinuria. After progressing to end-stage renal disease, each patient received a living-related renal transplant. Shortly after transplantation, proteinuria recurred in both patients, progressing to the nephrotic syndrome. Serial renal biopsies were obtained from each patient. These initially demonstrated focal segmental epithelial proliferation (the "cellular lesion"), but focal segmental scars were observed in subsequent biopsies. None of the biopsies demonstrated immunoglobulin, complement deposition, or diffuse epithelial cell foot process fusion. These findings suggest that the scarring lesion in recurrent FSGS may be the result of a primary process involving damage to a limited number of visceral epithelial cells.     

Usefulness of Plasma Exchange in Recurrent Nephrotic Syndrome Following Renal Transplant

Abstract: Nephrotic syndrome due to focal segmental glomerulosclerosis (FSGS) frequently recurs even after transplantation and may cause failure of the renal al-lograft. We report a case in which plasma exchange (PE) was used to treat a 32-year-old patient with biopsy-proven recurrence of FSGS in a second renal transplant after the first allograft had failed. One year after the second renal graft, the patient presented with proteinuria of 5 g/day and a creatinine level of 1.46 mg/dl. A course of 9 PE was performed over a 15-day period. Proteinuria improved rapidly, and 20 days after the last PE, it was already down to 0.8 g/day. This level continued to decrease progressively (0.5 g/day after 3 months) and reached zero after approximately 6 months. Eleven months after PE treatment, the patient had no proteinuria and his plasma creatinine level was normal. In our opinion PE should be instituted early in recurrent nephrotic syndrome after renal transplantation. The optimum frequency of such treatment still has to be established, especially with regard to its use as long-term maintenance therapy.     

Abrogation of nephrotic proteinuria by rituximab treatment in a renal transplant patient with relapsed focal segmental glomerulosclerosis

Relapse of focal segmental glomerulosclerosis (FSGS) after renal transplantation is 20-40%. Recurrence after a first relapse is 80%. The only current treatment is plasmapheresis and/or cyclophosphamide. We report successful treatment of a second relapse in a 48-year-old patient. At age 33, FSGS was diagnosed. The patient began hemodialysis 1 year later. In her first renal transplant, she developed recurrent FSGS and reached terminal transplant failure 3 years later. Eight years later, a second transplant was performed. Immunosuppressive regimen: steroids, mycophenolate mofetil (MMF), tacrolimus (TAC), and rabbit anti-thymocyte globulin. Proteinuria of 2-6 g/day was detected and a biopsy showed recurrent FSGS. Plasmapheresis was started without success. Another biopsy still showed FSGS. The patient received two doses of rituximab (375 mg/m2 each) i.v. Three weeks later, proteinuria was 350 mg/day (serum-creatinine 1.6 mg/dl). Twelve months later, proteinuria was at 90 mg/day. Rituximab might be an option for recurrent FSGS after renal transplantation.     

Tacrolimus in steroid-resistant and steroid-dependent nephrotic syndrome

BACKGROUND: Steroid resistance and steroid dependence constitute a major problem in the treatment of minimal-change disease and focal segmental glomerulosclerosis (FSGS). Cyclophosphamide and cyclosporine are well-established alternative immunomodulating agents, whereas data on FK 506 (tacrolimus) are rare. METHODS: The present work provides data from 10 patients of an open, monocentric, non-randomized, prospective trial. Five patients with steroid-dependent minimal-change nephrotic syndrome, 1 patient with steroid-refractory minimal-change disease and 4 patients with steroid-refractory FSGS were started on tacrolimus at trough levels of 5 10 microg/l. In case of steroid-dependence, prednisolone was tapered off in presence oftacrolimus within one month. RESULTS: Within 6 months, complete remission was achieved in 5 patients (50%) and partial remission in 4 patients (40%), yielding a final response rate of 90%. One patient was primarily resistent to tacrolimus (steroid-refractory minimal-change), another patient became secondarily resistant to tacrolimus after an initial remission (steroid-refractory FSGS). Average proteinuria significantly decreased by 77% from 9.5 +/- 1.4 - 2.2 +/- 1.1 g/day (p < 0.01). Serum protein significantly raised from 55.0 +/- 1.9 - 64.6 +/- 1.9 g/l (p < 0.01). Tacrolimus induced non-significant increases of blood glucose (4.9 +/- 0.1 - 5.1 +/- 0.2 mmol/l), systolic blood pressure (131.4 +/- 7.1 - 139.0 +/- 7.6 mmHg) and creatinine (93.2 +/- 13.9 103.2 +/- 15.3 mmol/l). Five patients have been tapered off tacrolimus so far, nephrotic syndrome relapsed in 4 of them (80%). Relapse occurred at tacrolimus levels between 2.6 and 6.9 ng/ml. CONCLUSIONS: Our data suggest that tacrolimus may be a promising alternative to cyclosporine both in steroid-resistant and steroid-dependent nephrotic syndrome.     

Intravenous cyclosporine therapy in recurrent nephrotic syndrome after renal transplantation in children

BACKGROUND: Early recurrence of massive proteinuria after renal transplantation occurs in 20% to 30% of patients with steroid-resistant idiopathic nephrotic syndrome and is responsible for graft failure in approximately half of cases. We report our experience with the use of intravenous (IV) cyclosporine (CsA) in children with recurrent proteinuria after renal transplantation. METHODS: Between March 1991 and August 2001, 36 renal transplantations were performed in 35 patients with steroid-resistant idiopathic nephrotic syndrome in our institution. Recurrence, defined by proteinuria higher than 50 mg/kg per day in the absence of acute rejection or urinary tract infection, was observed in 17 grafts performed in 16 patients. In patients with recurrence, CsA was administered IV, at an initial dose of 3 mg/kg per day, which was afterward adapted to maintain whole-blood levels between 250 and 350 ng/mL. RESULTS: In 14 of 17 cases (82%) with recurrence, proteinuria completely disappeared after 20.8+/-8.4 (range 12-40) days. The treatment was ineffective in the remaining three patients with persistent proteinuria at the end of the second month posttransplantation. Plasma exchanges were performed in four patients during the first 2 months, and proteinuria regressed in three cases and persisted in one. Persistent remission was observed in 11 patients with a follow-up of 3.7+/-3 (range 0.3-9) years. Actuarial graft survival was 92% and 70% at 1 and 5 years. CONCLUSION: IV CsA is a safe and effective treatment in children with recurrent nephrotic syndrome after renal transplantation.     

Severe renal complications in chronic myelomonocytic leukemia

A 56-year-old man presented with peripheral monocytosis and massive nephrotic syndrome. He was diagnosed as having chronic myelomonocytic leukemia and membranous glomerulonephritis. He received prednisone, chlorambucil and hydroxyurea, but the nephrotic syndrome persisted. Seven months after diagnosis, he was started on cyclosporin A; 1 month later he developed acute renal failure due to radiolucent bilateral renoureteral stones. His kidney function recovered after placing ureteral catheters and urine alkalization. Afterward, he was given mycophenolate mofetil, and proteinuria decreased to subnephrotic levels (1 g/24 hours). This case highlights 2 severe renal complications in this type of leukemia. To the best of our knowledge, there are only 2 previous cases of glomerulonephritis, histologically proven, associated with chronic myelomonocytic leukemia. On the other hand, reversible acute renal failure due to radiolucent bilateral renoureteral stones has never been reported. Also, as far as we know, mycophenolate mofetil was successfully used here for the first time for treating glomerulonephritis-related chronic myelomonocytic leukemia.     

Serum glomerular permeability activity in patients with podocin mutations (NPHS2) and steroid-resistant nephrotic syndrome

A plasma factor displaying permeability activity in vitro and possibly determining proteinuria has been hypothesized in idiopathic focal segmental glomerulosclerosis (FSGS). In vitro permeability activity (P(alb)) was determined in sera of five patients with autosomal recessive steroid-resistant nephrotic syndrome (NPHS2), an inherited condition indistinguishable from idiopathic FSGS on clinical grounds, but in which proteinuria is determined by homozygous mutations of podocin, a key component of the glomerular podocyte. All patients had presented intractable proteinuria with nephrotic syndrome; four developed renal failure and received a renal allograft. For comparison, sera from 31 children with nephrotic syndrome were tested. Pretransplant P(alb) was high in all cases (mean 0.81 +/- 0.06), equivalent to levels observed in idiopathic FSGS. Overall, P(alb) did not correlate with proteinuria. The posttransplant outcome was complicated in two patients by recurrence of proteinuria after 10 and 300 d, respectively, that responded to plasmapheresis plus cyclophosphamide. P(alb) levels were high at the time of the recurrence episodes and steadily decreased after plasmapheresis, to reach normal levels in the absence of proteinuria after the seventh cycle. In an attempt to explain high P(alb) in these patients, putative inhibitors of the permeability activity were studied. Coincubation of serum with homologous nephrotic urine reduced P(alb) to 0, whereas normal urine did not determine any change, which suggests loss of inhibitory substances in nephrotic urine. The urinary levels of the serum P(alb) inhibitors apo J and apo E were negligible in all cases, thus suggesting that other urinary inhibitors were responsible for the neutralizing effect. These data indicate that P(alb) is high in NPHS2, probably resulting from loss of inhibitors in urine. Lack of correlation of P(alb) with proteinuria suggests a selective loss of inhibitors. As in idiopathic FSGS, proteinuria may also recur after renal transplantation in NPHS2 patients, and post-transplant proteinuria is associated with high P(alb). The relationship between elevated P(alb) and proteinuria in NPHS2 remains to be determined.     

Long-term outcome of renal transplantation in focal glomerulosclerosis

INTRODUCTION: Focal segmental glomerulosclerosis (FSGS) has a tendency to recur frequently after kidney transplantation. We evaluated 12 cases to examine the incidence and long-term outcomes of recurrent FSGS. MATERIALS AND METHODS: Twelve patients with renal failure caused by FSGS received kidney allografts from living related donors. Tacrolimus or cyclosporine was used in combination with prednisolone and azathioprine or mycophenolate mofetil. RESULTS: The mean graft survival was 87.4 +/- 46.8 months. The graft survival rates in FSGS recipients were at 1 year, 100%; 5 years, 79.6%; 10 years, 68.2%. Two out of four recipients experienced graft loss due to chronic rejection. The other two out of four recipients with graft loss displayed severe proteinuria diagnosed as recurrence of FSGS. To treat recurrent FSGS, plasma exchange was partially effective to reduce proteinuria. CONCLUSION: Our incidence of recurrent FSGS is 16.7% with graft survivals at 5 and 10 years of 79.6% and 68.2%, respectively. The recurrence of FSGS happened after scheduled reductions in immunosuppressants. Careful observation is required with maintenance of immunosuppression in these patients.