狼疮肾炎终末期肾病患者尸体肾移植疗效观察

目的：探讨系统性红斑狼疮肾炎终末期肾病（LN-ESRD）患者肾移植的时机、疗效及术后狼疮活动情况。方法：回顾性分析16例LN-ESRD患者,女性14例,男性2例。年龄20～47岁,平均34.4岁。术前、术后狼疮活动指数（SLEDAI）,移植时机,移植后免疫抑制剂使用,人/肾存活情况。结果：16例患者术后10d内肾功能恢复正常。术后6个月内发生急性排斥3例（18.8%）,用甲泼尼龙（MP）冲击治疗后逆转。术前高血压10例,术后3个月后高血压7例,1例术后4个月因高血压脑出血死亡,当时无狼疮活动（SLEDAI〈10分）。15例至今仍存活,存活时间3～156个月,平均80.3个月）,其中有4例出现慢性移植肾肾病（1例重返透析治疗,3例肾功能不全,氮质血症期）,1例移植肾新发肾炎（病理检查为局灶、节段性硬化）,伴肾功能不全,氮质血症期。10例肾功能正常,其中1例出现少量蛋白尿。术前SLEDAI〈10分15例,SLEDAI〉10分1例（12分）,术后6个月后均无狼疮活动（SLEDAI〈10分）。本组患者定期追踪复查SLEDAI〈10分。结论：肾移植是治疗LN-ESRD有效的方法。狼疮无活动时,肾移植后系统性红斑狼疮复发率低。     

终末期狼疮肾炎患者进行肾移植的相关问题

狼疮肾炎（LN）是系统性红斑狼疮（SLE）最常见的并发症之一，约见于60％的SLE患者。尽管近年来LN的预后有了明显改善，但仍有5％-22％的患者发展为终末期肾病（ESRD），需行肾脏替代治疗。早在1975年，美国36个移植中心的汇总资料表明，LN移植肾存活率和患者2年生存率与其他ESRD患者无显著差别。此后终末期LN的肾移植迅速发展。迄今为止，几乎所有的研究均显示LN患者肾移植后病死率下降，5年生存率可大于90％，显示出LN患者行肾移植是可行的。     

狼疮肾病终末期患者肾移植的临床分析

目的：对6例系统性红斑狼疮（SLE）终末期肾病患者的肾移植情况进行总结。方法：对6例终末期狼疮肾病肾移植患者的临床、实验室资料和手术后随访情况进行分析。结果：6例患者均为女性，平均病程4.4年，进行移植手术时全身病情稳定；术前均使用激素和/或免疫抑制剂控制SLE病情，手术后免疫抑制剂使用情况差异无显著性，术后平均随诊时间为24.2个月，无1例发生急、慢性排异反应及狼疮肾病复发，移植肾功能良好。结论：终末期狼疮肾病患者的移植肾存活及功能状况良好，无狼疮肾病复发，肾移植是治疗终末期狼疮肾病的有效方法之一。     

高血压对狼疮肾炎预后的影响

目的　研究高血压对狼疮肾炎 (LN)肾功能损害发展的影响。方法　对 5 33例成年LN病人采用回顾性群体研究。以LN发展到终末期肾病 (ESRD)的时间为衡量指标。采用寿命表分析研究高血压对LN患者肾功能发展到ESRD的影响。结果　 5 33例患者平均随访时间为 7 8年 ,12 6例 (2 3 6 % )发展至ESRD。起病时伴随着高血压与ESRD的发展相关联。所有病例发展到ESRD的中位数时间分别为 ,重度高血压 (DBp >10 5mmHg) 8个月 ,轻度高血压 (DBp 90～ 10 4mmHg) 4 5个月 ,血压正常 112个月。多变量分析表明高血压独立影响着患者的预后 ,而患者的一般人口因素特点对ESRD的发展并无影响。结论　高血压是影响狼疮肾炎预后的重要的、潜在的可变因素。     

影响肾移植术后移植肾失功的因素

目的分析影响肾移植手术后移植肾失功的因素。方法接受同种异体肾移植手术的患者共750例,分析移植肾失功率及失功原因。应用Logistic分析影响移植肾失功的独立因素。结果 5年内移植肾失功率为3.87%,存活率为96.13%,肾移植受者的存活率为97.73%;移植肾功能延迟恢复、急性排斥反应、加速性排斥反应、感染,显著影响移植肾的存活率(P0.05)。年龄、免疫抑制方案对移植肾存活的影响无统计学意义(P0.05)。移植肾冷缺血时间、肌酐恢复正常时间、患者1个月内状况(移植肾功能延迟恢复、急性排斥反应、加速性排斥反应、感染)均属影响移植术后移植肾存活的独立因素。结论对肾移植术后患者进行移植肾功能延迟恢复、急性排斥反应、加速性排斥反应、感染的发生进行严格控制,对提高移植肾的存活率具有重要的临床意义。     

注射用霉酚酸酯预防移植肾急性排斥反应的多中心临床观察

目的：观察霉酚酸酯(MMF)注射剂与环孢素(CsA)和类固醇激素联合应用预防同种异体肾移植急性排斥反应的有效性和安全性。方法：免疫抑制方案为MMF(注射剂及口服剂)联合应用CsA和类固醇激素。观察肾移植术后3个月内急性排斥反应的发生率、程度、人／肾存活率、临床不良反应及感染发生情况。结果：所有受试者、移植肾均存活，82例受试者除1例为移植肾功能延迟恢复外，其余在移植术后3个月的血肌酐和尿量均满意，生命体征、症状和生活质量显著改善。有6例出现急性排斥反应，排斥率为7．32％，经相应治疗后均缓解。有15例受试者16次肝功能异常，经保肝治疗后均转为正常。1例血白细胞下降，经对症处理后缓解。药物相关不良反应发生率为20．73％。7例受试者出现机会感染，发生率为8.53％，经抗生素和抗病毒治疗后均痊愈。未见胃肠道副作用。结论：MMF注射剂有效，可预防肾移植术后急性排斥反应，特别适用于术后早期不能口服药物的肾移植患者。     

肾移植活体供肾的现状

自1954年Merrill等实施人类首例同卵双生兄弟间活体亲属供肾移植以来，肾移植技术，现已成为救治终末期肾脏病（ESRD）的最有效手段。特别在西方国家，活体供肾已被广泛应用。美国从2001年以来，活体供肾年移植量显然超过尸肾移植。在伊朗，自从1984年以来已做肾脏移植16000例，约95％以上的肾脏移植来源于活体供肾。与尸肾移植比较，活体供肾移植可获得更好的人／肾存活率，特别在一开始透析时就做活体肾移植的效果更好。透析时间0～6个月移植肾5年存活率78％，透析时间〉24个月者为58％；10年存活率分别为63％和29％。本文主要综述活体供肾移植的现状。     

霉酚酸酯对Ⅳ型狼疮肾炎治疗的临床观察并文献复习

目的 观察霉酚酸酯（MMF）对Ⅳ型狼疮肾炎（LN）的疗效。并对国内外MMF治疗LN的现况进行文献复习。方法 对传统治疗方法无效的10例Ⅳ型LN患者给MMF1－2g/d加中小剂量激素进行治疗。治疗时间最长者1年，最短者6个月，并在治疗后3、6、12个月进行动态观察。结果 治疗后尿蛋白逐渐减少，狼疮活动指数逐渐下降。治疗前后ANA阳性率为80％和30％，抗dsDNA抗体阳性率分别为50％和10％。低补体血症得以纠正，3例肾功能异常经治疗后2例恢复正常，治疗中仅发现少数病人有消化道反应，个别病人有带状疱疹。结论 MMF对Ⅳ型LN治疗作用确切，副作用小。     

抗中性粒细胞胞质抗体相关肾炎患者肾移植的转归

目的:回顾性分析抗中性粒细胞胞质抗体(ANCA)相关肾炎(AAGN)患者接受肾移植后的转归。方法:11例AAGN患者[男4例,女7例,中位年龄48(33.5~52.5)岁]接受肾移植。采用激素、吗替麦考酚酯联合他克莫司/环孢素A三联抗排斥治疗。肾移植后排斥反应及其类型均经移植肾活检病理诊断。回顾性分析移植肾预后、血管炎复发及血清ANCA对移植肾预后的影响。结果:11例AAGN均为MPO-ANCA相关血管炎,10例在肾移植前接受肾脏替代治疗,中位时间为30.5(14.8~50.5)个月。肾移植前均无血管炎活动(BVAS 0分),7例血清MPO-ANCA阳性。肾移植术后均未出现移植肾功能延迟恢复,4例(36.4%)发生急性细胞性排斥反应,分别有1例术后18个月和22个月发生血管炎复发和慢性体液性排斥反应。术后中位随访56(46.5~135)个月,随访末8例(72.7%)血清肌酐水平正常,2例血清肌酐升高,1例移植肾失功。5年人、肾生存率分别为100%和90.9%。术前ANCA阳性与阴性患者比较移植肾排斥反应发生率和血管炎复发率均无显著差异。结论:AAGN患者肾脏移植后远期预后好,血管炎复发率低,但术后早期急性排斥反应发生率高,应加强预防抗排斥反应。     

改良多靶点方案治疗60例难治性狼疮性肾炎近期疗效观察

目的提高难治性狼疮性肾炎（LN）的治疗水平。方法对60例难治性LN患者行改良多靶点方案（激素＋CTX＋他克莫司）治疗6个月,评价疗效,测定24 h尿蛋白定量、血沉（ESR）、血清白蛋白（Alb）、补体C3及狼疮活动指数（SLE-DAI）。6例行重复肾组织活检。结果治疗6个月后完全缓解30例,部分缓解24例,总有效率为91.7%。24 h尿蛋白定量、ESR及SLE-DAI均较治疗前明显降低,而血清Alb及补体C3均较治疗前显著升高。2例肾功能不全者SCr恢复正常。6例重复肾组织活检者病理改变均减轻。结论改良多靶点方案治疗难治性LN效果确切,但长期疗效有待进一步观察。     

Systemic lupus erythematosus in patients with chronic renal failure

The clinical courses of 36 patients with systemic lupus erythematosus (SLE) in whom chronic renal failure developed and who required dialysis for more than three months were studied. At the time dialysis was initiated, 14 of 36 patients (38.9 percent) had clinically active SLE, but only three of 24 (12.5 percent) had activity in subsequent years while receiving dialysis therapy. In the majority of patients, however, renal disease progressed to end-stage despite clinical quiescence of SLE. During the follow-up period (mean +/- SD, 36 +/- 39.8 months), eight patients died--six from infections and two from cardiac disease. Actuarial survival rates at one, two, and five years after dialysis treatment were 91.1, 78.8, and 68.9 percent, respectively. This study suggests that the progression of renal disease to end-stage in patients with SLE may be mediated by nonimmunologic mechanisms as well as SLE-related immunologic insults. In most of these patients undergoing long-term dialysis, SLE remains clinically inactive despite persistent serologic abnormalities. Survival of the patients undergoing dialysis is comparable with that of the general dialysis population.     

Analysis of lupus activity in end-stage renal disease treated by hemodialysis

OBJECTIVE: The activity of systemic lupus erythematosus (SLE) has been reported to decrease in patients who have developed end-stage renal disease (ESRD). However, extrarenal symptoms attributable to the disease activity are noted, especially during the first year of dialysis. We studied the clinical course and evaluate the disease activity of SLE in patients with ESRD on hemodialysis for more than 6 months. SUBJECT AND METHODS: Fourteen patients with SLE who had been initiated on maintenance dialysis at our center between 1982 and 1999 were examined retrospectively. Their clinical details, organ system manifestations, serologic profiles and immunosuppressive treatment regimens were reviewed. Patients with and without postdialysis flaras of SLE were compared statistically. RESULTS: Five patients exhibited 6 SLE flares under treatment with corticosteroids. Two flares occurred within the first year of the initiation of dialysis, and in 1 patient, aggravation of the disease activity was noted 98 months after the initiation of dialysis. Polyarthritis was noted in 5 cases and fever in 4 cases. The serum complement levels decreased in all 6 cases with relapse of SLE activity. Compared with the other 9 patients who did not exhibit SLE relapse, no significant differences were found in 5 patients who did with respect to the demographic and serologic features at the initiation of dialysis. CONCLUSION: We conclude that the disease activity does not always burn out in patients of SLE who show progression to ESRD. SLE flares can sometimes occur even after one year of the initiation of dialysis. SLE patients on dialysis should be carefully followed up by clinical and serological monitoring, and treated by appropriate immunosuppressive therapy.     

Systemic lupus erythematosus after renal transplantation: patient and graft survival and disease activity. The Dutch Working Party on Systemic Lupus Erythematosus

OBJECTIVE: To determine the outcome of renal transplantation in patients with systemic lupus erythematosus and end-stage renal failure and to compare disease activity after transplantation with disease activity before transplantation. DESIGN: Retrospective case finding using data for an 8-year period from the central registry for renal replacement therapy in The Netherlands. SETTING: Tertiary care hospitals with facilities for renal transplantation in the Netherlands. PATIENTS: Twenty-eight patients who fulfilled at least four of the American Rheumatology Association's criteria for the classification of systemic lupus erythematosus and who received a renal transplant. MEASUREMENTS: Actuarial survival rates for grafts and patients after transplantation, maximal nonrenal scores on the Systemic Lupus Erythematosus Disease Activity Index, and time-adjusted disease exacerbation rates in all patients before and after transplantation. RESULTS: The actuarial graft survival rate at 1 year and 5 years was 68% (95% CI, 47% to 82%) and 54% (CI, 25% to 77%), respectively, whereas the actuarial patient survival rate was 87% (CI, 69% to 96%) at 1 and 5 years. High disease activity was not found to affect graft survival adversely before the start of renal replacement therapy or during dialysis. After transplantation, disease activity per patient and the overall incidence of disease exacerbations decreased. One case of recurrent lupus nephritis was seen. CONCLUSIONS: Patients with systemic lupus erythematosus and end-stage renal failure are excellent candidates for renal transplantation; disease activity after transplantation is sporadic and low, and the recurrence of lupus nephritis is rare.     

Kidney transplantation in lupus patients: a case-control study from a single centre

This study was conducted to determine kidney transplantation (KTx) outcomes for Greek patients with renal failure caused by lupus nephritis (LN) compared with matched controls, kidney recipients with other causes of end-stage renal disease (ESRD). Twenty-six patients with systemic lupus erythematosus (SLE) subjected to 26 kidney transplants were studied. For comparative purposes a case-control group was selected, matched for gender, source of donor, age and time of KTx. Patient and graft survival estimates were calculated with the Kaplan-Meier product limit estimator and survival estimates were compared with the log-rank test. All patients received cyclosporine or tacrolimus in combination with azathioprine or mycophenolate mofetil for chronic immunosuppression in addition to steroids. Fourteen transplants were from living-related donors and 12 were from deceased donors. The graft survival rates for lupus patients were 88% at 1 year, 67% at 5 years, 38% at 10 years, poorer than the control survival rates of 92%, 92% and 84% (P=0.004). Patient survival in the lupus group did not differ from that of the controls. Survival in the lupus group was 92% at 1 year, 77% at 5 years and 77% at 10 years vs. 96%, 92% and 92% (P=0.26). Chronic allograft nephropathy was the major cause of graft loss. Recurrent LN was detected in two patients, but only one lead to graft failure. SLE patients compared with controls had significantly higher rates of hypertension, cardiovascular disease, infections and malignancies. Compared with matched controls, SLE patients had inferior but still satisfactory graft survival rates, whereas patient survival rates were similar.     

Renal transplantation in scleroderma

Although the outcome of renal transplantation in patients with systemic lupus erythematosus (SLE) has been studied, there are few reports about the outcome of patients with systemic sclerosis who have undergone renal transplantation. We retrospectively collected data from the United Network for Organ Sharing (UNOS) Scientific Renal Transplant Registry from a 10-year period. From 1987 to 1997, 86 patients with systemic sclerosis who had renal transplantation were identified. Of these 86 patients, 70% were women, 86% were Caucasian, and the mean age at transplantation was 50.4 years. The overall mortality was 24% of the patient group; 44% (38/86) of renal grafts failed. First- through fifth-year graft survival rates were 62%, 60%, 57%, 50%, and 47%, respectively. The causes of graft failure could not be ascertained in 24 of 38 patients (63%). Among the known causes, 5 had acute rejection, 4 had chronic rejection, 3 had recurrence of scleroderma, and 1 each had infection and graft thrombosis. Immunosuppressive regimens used in the patients with systemic sclerosis consisted of antilymphocyte globulin in at least 25%. Sixty percent received a combination of steroids, azathioprine, and cyclosporine. The use of cyclosporine was not associated with either improvement of graft survival or an increased rate of graft failure. Graft survival at 5 years in patients with systemic sclerosis was comparable to that of patients with SLE who received renal transplantation, according to existent medical literature. Based upon these data, renal transplantation is as effective a treatment for restoring renal function in patients with systemic sclerosis as it is in patients with SLE. Those patients with systemic sclerosis whose renal function did not improve with angiotensin-converting enzyme (ACE)-inhibitor treatments after scleroderma renal crisis should be considered as transplant candidates. Although the data are incomplete, the use of cyclosporine may not confer the advantage of improving graft survival in patients with systemic sclerosis as compared with SLE patients.     

Survival and specific outcome of sickle cell disease patients after renal transplantation

The prognosis of sickle cell disease (SCD) patients who need dialysis is poor, but experience with kidney transplantation is limited. This study assessed the characteristics of 36 SCD patients undergoing renal transplantation. Immediate post-surgical complications occurred in 25% of cases. Cytomegalovirus and bacterial infections were frequently observed. Twelve patients died after a median follow-up period of 17·4 months. Overall patient survival was significantly lower in SCD than in the control group without significant difference for overall death-censored graft survival. Our data suggest that renal transplantation should be systematically considered in SCD patients with end-stage renal disease.     

Systemic lupus erythematosus: analysis of disease activity in 55 patients with end-stage renal failure treated with hemodialysis or continuous ambulatory peritoneal dialysis. Dutch Working Party on SLE

PURPOSE: To compare disease activity in patients with systemic lupus erythematosus (SLE) (1) before and after the onset of end-stage renal failure and (2) during hemodialysis and continuous ambulatory peritoneal dialysis (CAPD). PATIENTS AND METHODS: Records of 55 patients with SLE currently being treated with dialysis were reviewed. Disease activity was measured according to the SLE Disease Activity Index, event rates per 1,000 months' patient observation, and use of medication. RESULTS: In the majority of patients, deterioration of renal function was slowly progressive over more than 2 years. After the initiation of dialysis for end-stage renal failure, maximal extrarenal disease activity and use of medication decreased markedly, but event rates for specific nonrenal manifestations of lupus did not decrease. Overall survival with dialysis was 89% after 5 years. During dialysis no difference was found in disease activity and use of medication between treatment with either hemodialysis of CAPD. Thrombocytopenia and elevated levels of anti-double-stranded DNA, however, occurred more frequently during CAPD. CONCLUSION: Patients with SLE have excellent survival rates with dialysis; their disease activity is diminished during dialysis but not abolished. No difference in survival or disease activity was found between patients undergoing hemodialysis or CAPD.     

Trasplante renal en lupus eritematoso sistémico: comparación de la supervivencia del injerto con otras causas de enfermedad renal terminal

Introduction

End-stage renal disease (ESRD) due to lupus nephritis (LN) occurs in 10%-30% of patients. Initially systemic lupus erythematosus (SLE) was a contraindication for kidney transplantation (KT). Today, long-term graft survival remains controversial. Our objective was to compare the survival after KT in patients with SLE or other causes of ESRD.

Recurrent focal glomerulosclerosis: natural history and response to therapy.

PURPOSE: Recurrent focal glomerulosclerosis (FGS) has been well documented since it was first reported in 1972. However, the course of the disease after transplantation and the optimal treatment regimen have not been well defined since the introduction of newer treatment modalities. PATIENTS AND METHODS: We reviewed all the charts of patients with biospy-proven FGS who received renal transplants at our institution from January 1980 through December 1990. Case histories consistent with diagnoses other than primary FGS (such as reflux nephropathy or intravenous drug use) were eliminated from the study. During this time period, 78 allografts were received by 71 patients with FGS. Independent variables that were analyzed included sex, race, time in months between the diagnosis of FGS and end-stage disease (dialysis or transplantation), age at time of transplantation, type of dialysis, source of allograft (cadaveric or living related), haplotype matching, donor-specific transfusions, age and sex of the donor, post-transplantation acute tubular necrosis, rejection episodes, immunosuppression regimen, use of plasmapheresis and angiotensin converting enzyme (ACE) inhibitors, and outcome. RESULTS: FGS recurred in 25 allografts (32%) of 21 patients. Biopsy-proven diagnosis of recurrence was made a mean of 7.5 months (range: 0.5 to 44 months) after transplantation. Patients who had rapid progression to end-stage disease tended to experience more frequent recurrences. Of seven patients who received a second transplant, five patients lost the first graft to recurrent FGS, and four of those patients (80%) had a recurrence in the second allograft. Recurrent disease developed in 34% of patients concurrently treated with cyclosporine and in 28% of those treated with prednisone and azathioprine alone (NS). Patients with recurrent FGS who were treated with ACE inhibitors benefited from a significant reduction of proteinuria. Six patients underwent plasmapheresis after diagnosis of the recurrence. Three of five patients in whom the diagnosis was made early in the course of the disease and in whom plasmapheresis was initiated immediately had reversal of epithelial foot process effacement and remission of proteinuria. End-stage disease eventually developed in 14 allografts (56%) an average of 23.7 months (range: 1 to 65 months) after diagnosis of recurrent disease. The cause of failure was chronic rejection in four allografts and recurrent disease in the remaining 10 allografts. CONCLUSIONS: FGS recurs in approximately 30% of allografts and causes graft loss in half of these. Patients who have lost a first allograft to recurrent FGS are at high risk for developing recurrent disease in a second allograft. Prolonged allograft survival is possible in patients with recurrent FGS and may best be obtained with a combination of treatment modalities including cyclosporine (perhaps in higher dosages than are routinely used in clinical renal transplantation), ACE inhibitors, and early use of plasmapheresis. The efficacy of these modalities supports the notion that recurrent FGS is caused by a circulating humoral mediator.     

Outcomes of kidney transplantation in patients with systemic lupus erythematosus: a single-center study

INTRODUCTION. This study aimed to compare outcomes of kidney transplantation in patients with systemic lupus erythematosus (SLE) and a matched control group of non-SLE kidney recipients. MATERIALS AND METHODS. In a case-control study, 33 patients with kidney transplantation due to end-stage renal disease caused by SLE were matched to a control group consisted of 33 non-SLE patients who had been transplanted during the same period of time in our center. The clinical characteristics, complications, and patient and graft survival were compared between the two groups. RESULTS. In each group, 12 patients (36.4%) received a kidney from a deceased donor, 15 (45.4%) from a living unrelated donor, and 6 (18.2%) from a living related donor. There was no significant difference between the outcome in SLE patients and duration of dialysis before transplantation. The mean duration of hospital stay was 23.4 ± 18.1 days in the SLE group, while it was 13.0 ± 7.3 days in the controls (P = .006). One-year graft survival was 79.0% in patients with SLE and 90.9% in non-SLE patients (P = .17). One-year patient survival was 93.9% in patients with SLE versus 81.8% in the controls (P = .26). Nine patients in the SLE group versus 11 patients in the control group developed posttransplant complications (P = .59). CONCLUSIONS. Although hospital stay after transplantation was longer in the SLE kidney recipients than controls, safety of kidney transplantation was comparable. Graft failure in the SLE patients was not significantly different between patients with different sources of kidneys.