BK virus infection in kidney transplant recipients

INTRODUCTION: Nephropathy associated with the polyomavirus type BK virus (BKV) has emerged as a cause of allograft failure linked to immunosuppressive regimens containing tacrolimus or mycophenolate mofetil (MMF). The outcome in BKV nephropathy is generally unfavorable, namely 50% of patients lose graft function. We herein report nine cases of BKV nephropathy after kidney transplantation. METHODS: From October 1998 to May 2003, 138 of 169 consecutive kidney transplant patients received tacrolimus-based immunosuppression, and 31 received cyclosporine-based immunosuppression. Additionally, 88.2% of the patients received mycophenolate mofetil (MMF). The diagnosis of BK infection was made by the presence of decoy cells in the urine and by allograft biopsy. RESULTS: There were nine cases of BKV nephropathy in kidney transplant recipients, an incidence of 5.3%. All patients with BKV nephropathy received tacrolimus, MMF, and steroids. The median time to diagnosis of BKV infection was 7.8 months after transplantation. All patients experienced an elevated serum creatinine, which stabilized or decreased in seven patients with altered or decreased immunosuppression. After a mean follow-up of 11.1 months, 2 (22.2%) of nine patients lost the graft. CONCLUSION: Because BKV nephropathy is a rare but serious complication after kidney transplantation, it should be included in the clinical differential of transplant dysfunction. In the absence of documented antiviral treatment, early diagnosis and judicious use of immunosuppressive agents is indicated to minimize the occurrence of BKV infection.     

Mycophenolate mofetil in primary glomerulopathies

Mycophenolate mofetil is an immunosuppressive agent that blocks purine biosynthesis, inhibits T and B-lymphocyte and mesangial proliferation. Mycophenolate mofetil is not nephrotoxic like calcineurin inhibitors and is widely used in solid-organ transplantation. Recently, mycophenolate mofetil has been introduced in the treatment of autoimmune diseases and primary glomerulopathies. This review analyzes the literature currently available on the treatment of primary glomerulopathies with mycophenolate mofetil. Encouraging results have been obtained in minimal change nephropathy where it may help to reduce the use of steroids in these patients who are often very young. The results obtained in medium and high risk patients with focal segmental glomerulonephritis and idiopathic membranous nephropathy were less encouraging. Conflicting results have been reported on IgA nephropathy in controlled trials. None of these studies attained level A evidence, meaning that randomized control trials of sufficient statistical significance are necessary to estimate the real effectiveness of mycophenolate mofetil in primary glomerulopathies.     

Recovery of native renal function after kidney transplantation

IgA nephropathy is the most frequently occurring glomerulonephritis in the developed world. It is treated in the terminal phase via dialysis and transplantation. Posttransplantation recurrence is common, but evolves slowly. Herein, recovery of native kidney function 10 years after transplantation in a patient with IgA glomerulonephritis is described, and the potential effect of immunosuppression therapy, in particular with mycophenolate mofetil, is discussed.     

Kinetics of cellular and humoral immunity in a successful case of positive crossmatch kidney transplantation: a case report

A positive crossmatch remains one of the major barriers to successful kidney transplantation. Highly sensitized patients are at greater risk of hyperacute rejection and subsequent graft loss after transplantation. Although recent advances in desensitization therapy allow kidney transplantation in these patients, the success rate is quite low. Herein, we have reported a successful case of positive crossmatch living donor kidney transplantation using a desensitization protocol with an immune monitoring assay.A 42-year-old woman with end-stage renal disease due to IgA nephropathy had been on hemodialysis for 36 months. She showed positive T-cell and B-cell cytotoxic crossmatches with her husband owing to pretransplantation blood transfusions. We performed a preconditioning regimen comprising a single dose of rituximab (375 mg/m²) combined with double-filtration plasmapheresis (DFPP) followed by low doses of intravenous immunoglobulin (DFPP/IVIG treatment). Tacrolimus (target trough level, 5-10 ng/mL) and mycophenolate mofetil (1500 mg/body) were started 2 weeks before the DFPP/IVIG treatment. After 6 DFPP/IVIG sessions, the crossmatch became negative. An induction quadruple immunosuppression protocol included tacrolimus, mycophenolate mofetil, basiliximab, and methylprednisolone. After the transplantation, the patient's immune status was evaluated regularly by mixed lymphocyte reactions (MLR) using an intracellular carboxyfluorescein diacetate succinimidyl ester (CFSE)-labeling technique (CFSE-MLR assay) and immunosuppressant therapy was adjusted accordingly. During the observation period, neither antibody-mediated rejection nor acute cellular rejection was encountered in this patient.     

来氟米特联合甲泼尼龙治疗IgA肾病的疗效观察

目的评价来氟米特联合甲泼尼龙治疗IgA肾病的疗效和安全性。 方法收集2008年12月至2016年10月在中国人民解放军总医院肾脏病科诊断为IgA肾病的患者75例,分别观察吗替麦考酚酯联合甲泼尼龙(MMF组,44例)与来氟米特联合甲泼尼龙(LEF组,31例)治疗IgA肾病的疗效和安全性。 结果两组患者基线资料无统计学差异(P>0.05)。分别治疗2、6、10个月之后,患者24 h尿蛋白定量显著减低,血清白蛋白水平得到明显改善。但两组间的2、6、10个月后的缓解率(20.45% vs 29.03%、70.45% vs 77.42%、72.73% vs 83.87%)和完全缓解率(9.09% vs 6.45%、38.64% vs 45.16%、40.91% vs 48.39%)、24 h尿蛋白定量、血清白蛋白、肌酐、复发率[4例(9.09%) vs 1例(3.23%)]以及不良反应[6例(13.64%) vs 4例(12.9%)]无统计学差异的意义(P>0.05)。 结论来氟米特联合甲泼尼龙与吗替麦考酚酯结合甲泼尼龙疗效相当,是治疗IgA肾病的安全有效方案之一。     

Kidney retransplantation following graft loss to polyoma virus-associated nephropathy: an effective treatment option in simultaneous pancreas and kidney transplant recipients.

Polyomavirus-associated nephropathy (PVAN) has emerged as an important cause of graft loss following kidney transplantation. Experience with kidney retransplantation (reKT) in PVAN is very limited, especially in the setting of uninterrupted immunosuppression protecting the still functioning pancreatic graft after simultaneous pancreas/kidney transplantation (SPK). We present a review of five cases of reKT in four SPK recipients with Type 1 diabetes mellitus from a single centre (a second reKT was performed in one patient following first reKT failure due PVAN recurrence). Pre-emptive nephrectomy of the failed graft was performed in three of the cases and all kidney grafts for reKT were harvested from cadaveric donors. All patients are dialysis- and insulin-independent at 30 (9-55), median (range), months following last reKT with maintenance immunosuppression consisting of tacrolimus/sirolimus in three and cyclosporine A/mycophenolate mofetil in one patient. In conclusion, reKT represents an effective treatment option in SPK patients with kidney failure on account of PVAN. Use of interventions designed to reduce active viral replication, including pre-emptive nephrectomy of the failed graft, should be considered before reKT.     

Mycophenolate mofetil restores renal function and spares steroids during idiopathic nephrotic syndrome in children. A cohort study

Renal function evolution during idiopathic nephrotic syndrome depends on treatment toxicity. Cyclosporin is effective as a steroid-sparing agent but patients are dependant on this drug, which can lead to renal toxicity. Mycophenolate mofetil, a widely used drug in organ transplantation, has short-term beneficial effects in glomerular diseases, including idiopathic nephrotic syndrome. Little is known about mycophenolate mofetil in children and long-term evolution. We analysed a cohort of 12 children with steroid-dependant nephrotic syndrome due to minimal change disease in remission with cyclosporine therapy. They were switched to mycophenolate mofetil, when renal toxicity was diagnosed. We evaluated the number of relapses, tolerance of this new treatment, renal function and body mass index under mycophenolate. After a follow-up of 31.25 months, mycophenolate mofetil alone was effective in preventing relapses in eight patients, without side effects. Renal function significantly improved and the final body mass index decreased. Three patients relapsed on discontinuation of mycophenolate mofetil. The results suggest that mycophenolate mofetil is effective and safe in preventing relapses in steroid-dependant nephrotic syndrome. Furthermore, switching from cyclosporine to mycophenolate mofetil restores renal function. Therefore, mycophenolate mofetil might be considered as an alternative to cyclosporine, to preserve renal function and spare steroids during idiopathic nephrotic syndrome in children.     

BK virus-associated nephropathy in a kidney transplant recipient successfully treated with cidofovir, the first case in Japan

Abstract:   A 51-year-old female received a kidney transplant, donated by her husband. The patient was induced with tacrolimus, mycophenolate mofetil and prednisolone. After methyl predonisolone pulse therapy without biopsy, allograft biopsy on POD 160 showed severe tubulo-interstitial nephritis with intranuclear inclusions. Urine cytology also showed decoy cells. Blood PCR detected an increase of BK virus DNA. She was diagnosed as having BK virus-associated nephropathy . Reduction of tacrolimus and switching of mycophenolate mofetil to mizoribine were done. Serum Creatinin (sCr) still rose to 3.0 mg/dl with persistent viremia and viruria. From on POD 268, 0.25 mg/kg of cidofovir was administered intravenously every two weeks over about four months. Biopsy on POD 387 revealed the disappearance of tubulitis with intranuclear inclusions, and decoy cells also disappeared from urine cytology. BK virus DNA in the blood decreased under the threshold level. sCr was stable and remained about 2.2 mg/dl for three months after the final treatment of cidofovir.     

Recurrent IgA nephropathy after renal transplantation despite immunosuppressive regimens with mycophenolate mofetil.

BACKGROUND: Transplantation offers an excellent option for patients with immunoglobulin-A nephropathy (IgAN) with severe renal dysfunction. However, IgAN frequently recurs in allografts treated with azathioprine. We examined the impact of mycophenolate mofetil immunosuppression on recurrence of IgAN. METHODS: We reviewed the charts of patients transplanted for IgAN at our institution in the cyclosporin era. Patients were excluded from further analysis if follow-up was <12 months or if immunosuppression at engraftment did not include azathioprine or mycophenolate mofetil. Laboratory data, medications and allograft biopsy findings were compiled. RESULTS: 152 kidney transplantations met the study criteria. At engraftment, 61 allografts were treated with azathioprine and 91 with mycophenolate mofetil. By 3 years post-transplant, IgAN developed in six of 60 (10.0%) azathioprine-treated allografts and five of 62 (8.1%) mycophenolate mofetil-treated allografts (P = 0.76). Overall, 13 azathioprine-treated and seven mycophenolate mofetil-treated allografts showed recurrence. As expected in this retrospective study, the duration of observation was longer in the azathioprine group. The interval between engraftment and diagnosis of recurrent disease was also longer. Survival of allografts with recurrent IgAN was similar in the two groups. Survival of allografts with recurrent IgAN was worse than for allografts without recurrence or allografts transplanted into patients with non-IgAN renal failure. Neither switching azathioprine to mycophenolate mofetil nor using an angiotensin-converting enzyme inhibitor or angiotensin-II type 1 receptor blocker ameliorated the clinical course after a biopsy documented recurrent IgAN. CONCLUSIONS: Mycophenolate mofetil, compared with azathioprine, did not lessen the recurrence of IgAN or its clinical impact.     

胰肾联合移植术后的免疫抑制治疗

目的探讨胰肾一期联合移植(SPK)术后免疫抑制药物的合理应用。方法 2005年1月至2009年6月我中心完成9例SPK,其中男5例,女4例,均采用空肠引流方式。术后采用IL-2单克隆抗体诱导的四联免疫抑制方案:IL-2单克隆抗体(舒莱或赛尼哌)+他克莫司(FK506)+霉酚酸酯(MMF)+激素,并逐渐过渡至单用FK506维持治疗。回顾性分析以上9例患者围术期及长期随访情况。结果 9例手术均获得成功。除1例早期死亡外,其余8例患者术后1周内肌酐降至正常水平,术后停用胰岛素时间为(11.5±3.5)d,空腹血糖恢复至正常时间为(15.4±6.3)d。8例患者随访4～50个月,共发生移植肾急性排斥4例,1例患者在接受床边血液透析过程中并发心脑血管意外后家属放弃治疗,其余3例患者经抗胸腺细胞球蛋白(ATG)或激素冲击治疗后移植肾功能均逆转恢复,随访过程中未发现移植胰腺排斥。结论胰肾联合移植是治疗糖尿病合并终末期糖尿病肾病的有效方法,术后早期采用IL-2单克隆抗体诱导的四联免疫抑制方案并逐渐过渡至单用FK506维持治疗是安全的。     

Coexisting glomerular IgA deposition and IgG-kappa multiple myeloma

Multiple myeloma (MM) is a common malignancy that often results in many kinds of kidney injuries for the abnormal monoclonal immunoglobulin. Here, we present an IgG-kappa type MM case accompanied by renal IgA deposition combined with IgG-kappa. The patient was treated with prednisone plus mycophenolate mofetil, and got a satisfactory remission. Although it cannot be determined whether the IgA deposition was secondary to MM, this was the first report of coexisting mesangial proliferative nephritis with IgA deposition and IgG-kappa type MM.     

Simultaneous kidney-pancreas transplantation without antilymphocyte induction

BACKGROUND: The introduction of potent new immunosuppressive agents may allow simultaneous kidney-pancreas transplantation to be performed without antilymphocyte induction. METHODS: We analyzed 30 simultaneous kidney-pancreas transplantations receiving tacrolimus, mycophenolate mofetil, and steroids without without antilymphocyte induction. Eighteen patients underwent pancreas transplantation with portal-enteric (P-E) drainage and the remaining 12 had systemic bladder (S-B) drainage. Target 12 hr trough tacrolimus levels for the first 3 months after simultaneous kidney-pancreas transplantation were 15-20 ng/ml. The oral mycophenolate mofetil dose was 2-3 g/day begun immediately posttransplant in two to four divided doses. Steroids were tapered according to protocol. RESULTS: All patients experienced immediate function of both kidney and pancreas grafts. One-year actuarial patient, kidney, and pancreas graft survival rates are 93, 93, and 90%, respectively. Nine patients (30%) had a total of 13 rejection episodes (12 biopsy proven) including 4 within 2 weeks, 6 between 2 weeks and 3 months, and 3 beyond 3 months after simultaneous kidney-pancreas transplantation. Three rejection episodes were treated with steroids alone and 10 were treated with antilymphocyte therapy (5 OKT3 and 5 ATGAM). A total of seven patients (23%) received antilymphocyte therapy. Three patients (10%) had more than one rejection episode. Two pancreas grafts (7%) and one kidney graft (3%) were lost from rejection. Four patients (13%) developed cytomegalovirus infection, but none had tissue-invasive cytomegalovirus. At present, 22 surviving patients (81%) remain on triple immunosuppression with tacrolimus, mycophenolate mofetil, and prednisone with excellent dual graft function. CONCLUSION: Tacrolimus, mycophenolate mofetil, and prednisone immunosuppression without without antilymphocyte induction is safe and effective after simultaneous kidney-pancreas transplantation.     

Prevalence and clinical course of BK virus nephropathy in pancreas after kidney transplant patients

The influence of BK virus nephropathy (BKVN) in pancreas after kidney (PAK) transplantation is unclear. A retrospective analysis of PAK transplants performed at our center was conducted to determine the impact of BKVN. Among 40 PAK transplants performed using sequential immunosuppression, four patients developed BKVN, as defined by a >20% rise in serum creatinine and BK viremia (BK plasma load >4 log copies/mL), at a median of 19 months following PAK. In all four patients, treatment of BKVN consisted of reduction in tacrolimus, cessation of mycophenolate mofetil, and introduction of leflunomide. With this approach, two patients experienced improvement or stabilization of renal function. The remaining two patients progressed to dialysis dependence despite treatment. Plasma BK load < or =5 log copies/mL was associated with graft preservation. Gender, age, delay between transplants, cumulative Thymoglobulin dose, and type of kidney donor were not associated with BK virus infection. Pancreas graft rejection or dysfunction was not observed with the above immunosuppression modification. Mean amylase and lipase > or =6 months following BKVN treatment remained normal. BKVN is an important cause of kidney allograft loss in PAK patients. Screening and early treatment of BKVN may enable preservation of kidney and pancreas grafts.     

Renal transplantation for end-stage renal disease following bone marrow transplantation: A report of six cases, with and without immunosuppression

BACKGROUND. Over 12000 bone marrow transplantations (BMT) are performed in the USA each year. This procedure is associated with significant morbidity including acute and chronic renal failure (CRF). CRF after BMT is usually secondary to radiation nephropathy and,or cyclosporine (CsA) toxicity. Survival on dialysis therapy for patients with radiation nephropathy is poor and renal transplantation may be a preferable form of renal-replacement therapy. METHODS: We report our experience with renal transplantation in 6 patients with end-stage renal disease (ESRD) following BMT: 4 as a result of radiation nephropathy; one secondary to hemolytic uremic syndrome; and 1 as a result of antitubular basement membrane nephritis. Ages at the time of BMT ranged from 26 to 40 yr. ESRD developed after a mean period of 94 months (range 42-140 months) after BMT. The kidney source was from a living donor in 5 patients, and a cadaveric donor (CAD) in 1 patient. In 3 recipients, the bone marrow and kidney were from the same donor. They are managed without any immunosuppressive therapy. The other 3 were initiated on triple therapy (prednisone, mycophenolate mofetil/azathioprine and cyclosporine/tacrolimus). RESULTS: These patients have been followed for up to 31 months (range 3-30 months) after kidney transplant, and 5 out of 6 are alive with functioning bone marrow and renal transplants. Their plasma creatinines range from 70 to 160 micromol/L (mean 97 micromol/L). One patient died following metastatic squamous cell cancer of the genital tract. CONCLUSIONS: 1) Renal transplant is a feasible alternative for patients with ESRD following BMT: 2) if bone marrow and kidney are from the same donor, the recipient requires little or no maintenance immunosuppression; 3) short-term results show good survival, but long-term follow-up is needed: 4) infections and malignancy post-renal transplantation were seen in recipients who needed immunosuppression; and 5) reduction in immunosuppression may be needed in such post-BMT patients who undergo kidney transplants.     

3例多囊肾病合并原发性IgA肾病病例分析

目的常染色体显性遗传多囊肾病（autosomal dominant polycystic kidney disease,AD-PKD）发病率为1／1000-1／400,是主要由PKD1或PKD2基因突变而引起的遗传性肾病。ADPKD合并IgA肾病（IgAnephropathy,IgAN）的病例临床上较为少见,可伴有肾病综合征。本研究旨在探讨ADPKD合并原发性IgAN的病理特点和治疗方案。方法对3例ADPKD并IgAN患者的临床表现、ADPKD家族史、实验室检查、病理诊断及预后进行回顾性分析。结果3例患者发病年龄31-53岁,均以少尿、水肿、大量蛋白尿为主要症状,肾穿刺活检术后诊断为1例HassII型IgAN和2例HassI型IgAN。病例1给予泼尼松联合环磷酰胺治疗,病例2给予泼尼松联合吗替麦考酚酯治疗,病例3单用泼尼松治疗。经过免疫抑制治疗后,患者大量蛋白尿和血尿均得到缓解。虽然患者随访时总肾脏体积仍出现增长,但长期肾功能保持良好。结论ADPKD伴大量蛋白尿根据囊泡位置尽可能开展肾活检。ADPKD并IgAN的患者应根据分型给予循证支持的免疫抑制治疗,可以减少蛋白尿,有助于预防肾衰竭的发生。     

IgA nephropathy in a young man with primary hyperparathyroidism

We report the first documented case of IgA nephropathy occurring after treatment of primary hyperparathyroidism. A 29-year-old man with a history of kidney stones and primary hyperparathyroidism underwent kidney biopsy for persistent proteinuria and microhematuria 18 months after resection of an ectopic parathyroid adenoma with subsequent normalization of serum calcium and parathyroid hormone levels. On ultrasound, renal intraparenchymal calcifications were noted. Renal biopsy revealed IgA nephropathy in addition to tubulointerstitial microcalcifications. The development of IgA nephropathy may have been influenced by hyperparathyroidism and/or its treatment. The case highlights the role of renal biopsy in patients with a history of kidney stones and abnormal urinary findings.     

Renal transplant patients with gastrointestinal intolerability to mycophenolate mofetil: conversion to enteric-coated mycophenolate sodium

The introduction of mycophenolate mofetil (MMF) was an important advance in immunosuppressive therapy, although its use is limited by adverse gastrointestinal events. Enteric-coated mycophenolate sodium (EC-MPS; myfortic) has been developed to avoid these side effects. Recent clinical trials have demonstrated that EC-MPS is a safe drug in both de novo and maintenance renal transplant patients. In this prospective study, therapeutically equivalent doses of EC-MPS were administered to 39 stable kidney transplant patients receiving MMF. After 3 months of treatment with EC-MPS the incidence of adverse gastrointestinal events was lower (15.8% of the patients). There were higher levels of mycophenolic acid after conversion to EC-MPS, probably due to better absorption. These factors allowed decreased doses and levels of calcineurin inhibitors without increasing the risk of graft rejection. At 3 months postconversion, serum creatinine improved from the mean baseline value of 1.83 +/- 0.12 mg/dL to 1.70 +/- 0.10 mg/dL. In conclusion, EC-MPS was well tolerated in maintenance renal transplant patients with adverse gastrointestinal events secondary to MMF.     

Mycophenolic acid therapy after cyclophosphamide pulses in progressive IgA nephropathy

BACKGROUND: In progressive IgA nephropathy (IgAN), cyclophosphamide or steroids have been used to reduce the loss of renal function, but disease progression may occur after the end of treatment. The value of mycophenolic acid (MPA) maintenance therapy following initial immunosuppression in progressive IgAN is largely unknown. METHODS: In a prospective single-center trial, 20 patients with advanced IgAN (median glomerular filtration rate [GFR], 22 ml/min) and disease progression after cyclophosphamide pulse (CyP; n=18) or steroid pulse therapy (n=2) were treated with MPA for a median of 27 months. MPA dosages (initially mycophenolate mofetil 500 mg twice daily) were adjusted according to predose plasma concentrations (target concentrations 1.5 to 4 microg/mL). The course of renal function was assessed by linear regression of glomerular filtration rates. RESULTS: Median loss of renal function per month was significantly reduced from -0.8 ml/min to -0.03 ml/min per month after 6 months, to -0.05 ml/min per month after 12 months, and to -0.12 ml/min per month at the end of the study after median 27 months (p<0.05). An improved or stable GFR was observed in 16 of 20 patients during the first 12 months, and sustained in 10 patients during 24 months of follow-up. Proteinuria decreased significantly from 1.1 g/L to 0.4 g/L during MPA treatment (p=0.018). CONCLUSION: Our results indicate that MPA may be beneficial to slow down the loss of renal function in patients with progressive IgAN after previous immunosuppressive treatment.     

Chronic cyclosporine-induced nephrotoxiciy in heart transplant patients: long-term benefits of treatment with mycophenolate mofetil and low-dose cyclosporine

BACKGROUND: Cyclosporine-induced nephropathy is a major limitation in heart transplant patients. Cyclosporine dose reduction may lead to substantial early improvement in renal function. Our aim was to study the long-term benefits of therapy with low doses of cyclosporine plus mycophenolate mofetil in heart transplant patients with drug-induced nephrotoxicity. METHODS: Twenty-five adult heart transplant patients with cyclosporine-related nephrotoxicity (mean posttransplant = 41.7 +/- 25.7 months) were included in the retrospective analysis (22 men, mean age = 58.8 +/- 7.9 years.). Patients were switched from azathioprine to mycophenolate mofetil (1 to 3 g/d), followed by a stepwise reduction in cyclosporine dosage (trough cyclosporine level maintained around 100 ng/mL). Renal function was determined by serial measurements of serum creatinine and glomerular filtration rate at 3-month intervals. RESULTS: With a mean follow-up of 30 +/- 13 months, the baseline creatinine of 2.37 +/- 0.5 mg/dL decreased to 1.59 +/- 0.40 mg/dL (P < .0001). The baseline glomerular filtration rate of 36.77 +/- 10.10 mL/min improved to 54.98 +/- 13.80 mL/min (P < .0001). The cyclosporine level was the unique independent variable associated with renal functional improvement (partial R(2) = 0.4). Within the first 3 months, renal function displayed a rapid improvement after conversion to mycophenolate mofetil (P < .001), reaching a plateau, without further significant improvement over the course of time. CONCLUSIONS: Cyclosporine-induced nephrotoxicity is not a progressive, irreversible disease. Reduction in cyclosporine exposure by addition of mycophenolate mofetil is useful to achieve long-term renal functional improvement, thereby avoiding chronic renal failure. A unique, significant factor associated with this improvement was the reduction in cyclosporine level.     

Treatment of IgA nephropathy

IgA nephropathy (Berger's disease) is the most common primary glomerulonephritis worldwide and was once equated with benign recurrent hematuria. Longer observation showed that 15% to 30% of patients progress to end-stage renal failure after 20 years of clinical manifestations. Because the pathogenesis remains enigmatic, therapy to ameliorate disease progression can not be disease-specific. Several approaches to treatment have generated increasing interest in the last few years, including angiotensin inhibition, glucocorticoids, fish oil, cyclophosphamide, tonsillectomy and mycophenolate mofetil. For patients reaching end-stage renal failure, recurrent disease after transplantation remains a clinically important problem, despite immunosuppression since engraftment.