免疫抑制剂与原发性膜性肾病

原发性膜性肾病(IMN)是成人肾病综合征最常见的类型,在大多数的患者中发病缓慢且表现良性经过.其自发的肾病综合征完全缓解率和部分缓解率高达30%.尽管如此,仍然有30%～40%在5～15年中进展为终末期肾脏病.本文的目的在于评价免疫抑制剂在治疗原发性膜性肾病中的意义.     

成人特发性膜性肾病肾病综合征的治疗进展

成人特发性膜性肾病肾病综合征的治疗进展刘刚综述王海燕审校特发性膜性肾病（ＩＭＮ）是成人原发性肾病综合征（ＮＳ）的一种常见病理类型。但是，目前ＩＭＮ的病因、发病机理尚不清楚，其临床发展快慢不一，其中约２５％的患者临床表现可自缓解，还有２０％～４０％的患...     

中西医结合治疗原发性膜性肾炎肾病综合征的临床观察

目的：探讨中西医结合治疗原发性膜性肾炎肾病综合征的临床疗效。方法：对48例原发性膜性肾炎肾病综合征分为治疗组和对照组。治疗组24例，用强的松、环磷酰胺并辨证加用中药；对照组24例，单纯用西药治疗，观察临床缓解率和副反应。结果：治疗组完全缓解率为54．11％，总缓解率为91．6％；对照组完全缓解率41．6％，总缓解率66．6％。治疗组总缓解率高于对照组（P＜0．05）；治疗组副反应率25％，对照组为66．6％。治疗组副反应低于对照组（P＜0．01）。结论：中西医结合是治疗原发性膜性肾炎肾病综合征的有效方法。     

干燥综合征合并膜增生性肾小球肾炎1例

干燥综合征肾损害以肾小管间质损害为主,累及肾小球较少见。本文报告1例经唇腺和肾活检证实的原发性干燥综合征合并膜增生性肾小球肾炎病例。患者以大量蛋白尿起病,经激素联合羟氯喹、环孢素等药物治疗后病情好转。     

原发性膜性肾病的治疗

原发性膜性肾病常表现为肾病综合征 ,病变的特征是肾小球基膜出现多数钉突 (嗜银染色 ) ,肾小球毛细血管壁的上皮细胞下有沉积物。免疫荧光可见ＩｇＧ及Ｃ3。目前多数学者认为是一种原位免疫复合物形成致病。该病可见于任何年龄 ,但好发于中老年 ,男性多于女性。在成人原发性肾病综合征中 ,在国外约占 30 % ,在我国则不多 ,约占 1 0 %。80 %以上的病例有明显的肾病综合征表现 ,其余病例仅表现为单纯的蛋白尿 ,一般无血尿。本病的病程进展缓慢 ,通常持续蛋白尿多年 ,肾功能才逐渐恶化。肾病综合征可自发性缓解和复发交替。本病晚期才有高血压…     

叶任高教授中西医结合治疗膜性肾病型肾病综合征的临床经验

叶任高教授是我国著名肾脏病和中西医结合专家。现任中山医科大学内科教授、博士生导师，卫生部肾病重点实验室主任，中国中西医结合学会肾专业委员会主任委员。他幼承家学，业医四十余载，在肾脏病的中西医结合治疗方面做了大量开创性工作，享有国际声誉。 膜性肾病（ＭＮ）是成人肾病综合征（ＮＳ）的主要病理类型，发病高峰在３６岁～４０岁。大量蛋白尿是影响本型肾病预后的公认因素。目前多数学者认为本病疗效不佳，甚至于治疗其他病理类型ＮＳ常用的肾上腺皮质激素（简称激素）和环磷酰胺（ＣＴＸ）的使用也存在争议［１］。叶任高教…     

原发性肾病综合征合并高血糖性脱水综合征1例

原发性肾病综合征的诊断标准是：大量蛋白尿（≥3．5g／d，或≥3．5g·1．73m^-2·24h^-1），常伴有低蛋白血症（〈30g／L）；高脂血症和水肿。其中前两条为诊断的必要条件。临床工作中肾病综合征病人，疾病治疗过程中，因糖皮质激素导致的血糖升高后失治，最后形成高血糖性脱水综合征，现述病例如下。     

肾病综合征特发性膜性肾病患者临床病理特征与肾功能的相关性

目的:探讨肾病综合征特发性膜性肾病(IMN)患者临床病理特征与肾功能的相关性。方法:对2008年01月~2019年06月在上海中医药大学附属龙华医院肾病科就诊并经肾活检确诊为IMN的112例患者的临床及病理资料进行回顾性分析。结果:与CKD1期患者相比,CKD2期及CKD3~5期患者在年龄、高血压及高尿酸血症患病率、血尿酸水平、肾小球球性硬化、肾小管萎缩及间质纤维化、肾间质炎症细胞浸润和小动脉管壁增厚等临床病理表现上更为严重;Logistic二元逐步回归提示年龄、高血压及高尿酸血症发病率、肾小管萎缩及间质纤维化和肾间质炎症细胞浸润是eGFR <90 ml/min的独立危险因素。ROC曲线显示血尿酸对女性患者肾功能的预测优于男性。结论:年龄、高血压、高尿酸血症、肾小管萎缩及间质纤维化、肾间质炎症细胞浸润是影响伴肾病综合征IMN患者肾功能的重要因素,血尿酸水平对不同性别患者肾功能的影响可能存在差异。     

原发性干燥综合征合并原发性胆汁性肝硬化患者骨密度分析

目的 回顾性分析原发性干燥综合征（primary sjogren''s syndrome,pSS）合并原发性胆汁性肝硬化（primary biliary cirrhosis,PBC）患者3D骨密度特点和骨折风险,并分析骨量变化与疾病活动度的关系。方法 选取2017-2021年于内蒙古科技大学包头医学院第一附属医院风湿免疫科诊断为pSS的患者64例,其中pSS合并PBC的患者33例,pSS未合并PBC的患者31例,两组患者的年龄及性别差异均无统计学意义。结果（1）pSS合并PBC组的腰椎二维骨密度（bone mineral density,BMD）较pSS未合并PBC组降低（P＜0.05）;（2）pSS合并PBC组股骨BMD较pSS未合并PBC组降低（P＜0.05）;（3）pSS合并PBC组股骨3D体积骨密度明显低于pSS未合并PBC组（P＜0.05）;（4）pSS合并PBC组患者的10年骨折风险明显高于pSS未合并PBC组的患者,两组相比差异有统计学意义;（5）pSS合并PBC组患者的胆红素水平与10年骨折风险、3D体积骨密度中的Inter Tro骨小梁有相关性（P＜0.05）;与3D体积骨密度中的Inter Tro骨皮质、Inter Tro全部及股骨BMD均无相关性（P＞0.05）;（6）pSS合并PBC组患者的血沉（ESR）、C-反应蛋白(CRP)、免疫球蛋白与股骨3D体积骨密度、10年骨折风险均无相关性（P＞0.05）;（7）pSS合并PBC组患者的谷丙转氨酶(ALT)、谷草转氨酶(AST)、r-谷氨酰转肽酶(GGT)、碱性磷酸酶(ALP)与股骨3D体积骨密度、10年骨折风险均无相关性（P＞0.05）。结论 pSS合并PBC患者比pSS未合并PBC患者骨量减少增加、进一步导致骨质疏松以及骨折的风险增加,为pSS合并PBC患者早期骨质疏松的防治提供理论依据。     

Preeclamptic nephropathy associated with membranous nephropathy

We report a patient who had nephropathy of toxemia of pregnancy associated with membranous glomerulonephritis, which deteriorated 3 months after delivery. Electron microscopy revealed electron-dense deposits on the subepithelial surface of the glomerular basement membrane and finely granular/fibrillar materials in the subendothelial space. As a result of treatment with prednisolone and anticoagulants, marked alleviation of both proteinuria and edema was achieved. We therefore consider that the change from subclinical to clinical membranous nephropathy could be attributed to pregnancy. Received: February 22, 2001 / Accepted: July 12, 2001     

Effect of cyclosporine therapy on idiopathic membranous nephropathy presented with refractory nephrotic syndrome

Background. Recent studies suggested the possible benefits of cyclosporine (CsA) therapy in patients with membranous nephropathy, although most of these studies were short-term. An uncontrolled retrospective study was undertaken to evaluate the long-term effect of CsA therapy on idiopathic membranous nephropathy presented with refractory nephrotic syndrome. Methods. The subjects were eight patients with idiopathic membranous nephropathy presenting with refractory nephrotic syndrome. All patients had received a course of corticosteroid therapy before CsA therapy, and had not responded to the corticosteroid, including one or two administrations of intravenous methylprednisolone pulse therapy. The CsA doses were adjusted to maintain trough blood level at 100 ng/ml during the first 3 months and then reduced to maintain the level at 50 ng/ml in patients who had responded to partial remission. Results. CsA therapy induced a marked decrease in proteinuria from the first month, and a significant decrease from month 3 and thereafter. The mean serum total protein and albumin levels rose, and total cholesterol fell significantly with CsA therapy. The serum creatinine level was unchanged during CsA therapy. Three patients showed complete remission and two were in partial remission, while three were nephrotic at 12 months of CsA therapy. From 18 to 24 months of CsA therapy, three patients were in complete remission, four were in partial remission, and one patient was nephrotic. There were no side effects of CsA, except for gum hyperplasia and hypertrichosis in one patient. Conclusion. These results suggest that long-term CsA therapy at a low or moderate dose is potentially effective and safe in most nephrotic patients with idiopathic membranous nephropathy refractory to corticosteroid therapy. Received: February 22, 1999 / Accepted: July 30, 1999     

Idiopathic membranous nephropathy in children

Idiopathic membranous nephropathy (MN) is a rare cause of asymptomatic proteinuria (AP) or nephrotic syndrome (NS) in childhood. To improve our understanding of its clinical course, we retrospectively reviewed 19 cases of idiopathic MN seen in our hospital over a period of 28.5 years, i.e., from January 1977 to July 2005. Eight patients (39%) had AP and 11 (61%) presented with NS. All eight AP patients achieved remission, regardless of treatment modality. Oral corticosteroid was given to all 11 NS patients, but only three of them responded to corticosteroid. Of the eight steroid non-responders, three achieved remissions with the addition of cyclosporine, and the five who were not administered additional immunosuppressive drugs had persistent NS. At the latest evaluation, all six NS patients that achieved remission remained free of proteinuria and had a normal renal function. Moreover, two of the 5 steroid non-responders showed persistent nephrotic-range proteinuria but a stable renal function. The remaining three steroid non-responders progressed into chronic renal insufficiency, and this progression was preceded by renal vein thrombosis (RVT) in two of the three patients. Presentation with NS (P=0.045) and the development of RVT (P=0.010) were identified as poor prognostic factors.     

Cytokine upregulation in tubulointerstitial nephritis associated with membranous nephropathy

We describe the upregulation of cytokines in a 45-year-old woman with tubulointerstitial nephritis and membranous nephropathy revealed by renal biopsy. She was treated with a combination of prednisolone and cyclosporin. Histological findings showed appreciable improvement, and urinary protein excretion was decreased from 15 g/day to 1 g/day. Elevated urinary levels of chemokines, interleukin (IL)-8 and monocyte chemotactic and activating factor (MCAF)/monocyte chemoattractant protein (MCP)-1, and serum levels of tumor necrosis factor (TNF)-α decreased during convalescence; 13 other patients with membranous nephropathy did not show elevation of these cytokines. These results suggest that the upregulation of these cytokines may participate in the pathogenesis of tubulointerstitial nephritis and that combination therapy of prednisolone and cyclosporin may be effective, possibly via inducing a decrease in these cytokines. Received: May 6, 1998 / Accepted: October 9, 1998     

Thrombotic complications in childhood-onset idiopathic membranous nephropathy

While the most common clinical feature of nephrotic syndrome is generalized edema, patients are at risk of developing other problems, such as bacterial infections, electrolyte abnormalities, and venous thromboses. Adults with membranous nephropathy appear to be at the greatest risk for developing thromboses, especially renal vein thrombosis. However, the same is not true for children with membranous nephropathy. A review of pediatric membranous nephropathy stated that renal vein thrombosis is unrecorded in childhood-onset membranous nephropathy. We present our experience in managing two children with idiopathic membranous nephropathy who developed venous thromboses. To our knowledge, this is the first report of pediatric patients with membranous nephropathy to develop a thromboembolic complication without evidence of predisposing factors or coagulation abnormalities. This report emphasizes the need for appropriate evaluation of patients with membranous nephropathy who develop signs and symptoms suggestive of arterial or venous occlusion in order to avoid missing this potentially life-threatening medical complication.     

Prognosis of lupus membranous nephropathy in children

The occurrence of membranous nephropathy in pediatric series of systemic lupus erythematosus has been reported only rarely, probably due to a very low frequency. One hundred fifty-four children who were seen in 100 French pediatric centers between January 2002 and April 2005 were included. Fifteen (12 girls and three boys) out of the 81 (18.5 %) children with renal involvement presented histological features of membranous nephropathy. Their ages ranged from six to 15 years old (mean=11.3) at the age of SLE diagnosis and 8/15 children were of African origin. Isolated membranous nephropathy was observed in nine patients, of whom five patients displayed a complete recovery following immunosuppressive treatment. Associated proliferative lesions were observed on the first kidney specimen in two patients and in a further renal biopsy in four other patients, leading to a less favorable course of lupus nephropathy.     

Acute kidney injury in idiopathic membranous nephropathy with nephrotic syndrome

Background and objectivesThe impact of acute kidney injury (AKI) on the progression of renal function in idiopathic membranous nephropathy (iMN) with nephrotic syndrome (NS) patients have not yet been reported, we sought to investigate the incidence, clinical features and prognosis of AKI in iMN with NS patients and determine clinical predictors for progression from AKI to advanced chronic kidney disease (CKD) stage.MethodsWe analyzed clinical and pathological data of iMN with NS patients retrospectively collected from Jan 2012 to Dec 2018. The primary renal endpoint was defined as persistent eGFR <45ml/min per 1.73 m² more than 3 months. Comparisons of survival without primary renal endpoint were performed by Kaplan-Meier curves and log-rank test. Univariate and multivariate Cox proportional hazard models were constructed to determine independent variables associated with primary renal endpoint .Results434 iMN with NS patients were enrolled. The incidence of AKI 1 stage, AKI 2 stage and AKI 3 stage was 23.1, 4.8 and 0.7% respectively. 66 (53.2%) patients with AKI had complete renal function recovery and 42 (33.9%) patients with AKI reached primary renal endpoint. Survival without primary renal endpoint was worse in AKI patients than No AKI patients (67.1 ± 5.3 and 43.7 ± 7.3% vs 99.5 ± 0.5 and 92.5 ± 4.2% at 2 and 4 years,p < 0.001). AKI was independently associated with primary renal endpoint, with an adjusted hazard ratio(HR) of 25.1 (95%CI 7.7–82.1, p < 0.001).ConclusionsAKI was usually mild and overlooked in iMN patients with NS, but it had a strong association with poor clinical outcomes and was an independent risk factor for CKD progression.     

Tiopronin-induced membranous nephropathy: a case report

Background: Tiopronin, a glycine derivative extensively used to treat cystinuria and hepatic cell injury, can give rise to rare complications such as proteinuria and nephrotic syndrome. However, the pathological characteristics of this secondary nephropathy are poorly understood. Here, we report a case of tiopronin-induced nephrotic syndrome. Case presentation: A 65-year-old Chinese man with a history of myasthenia gravis admitted tiopronin for hepatoprotection therapy. After 3 months later, he presented with rapid weight gain, massive peripheral edema, and proteinuria in the nephrotic range. Laboratory findings included serum albumin (20?g/L), total protein (38?g/L), and total cholesterol (11.78?mmol/L). A 24-hour urine protein collection contained 8620?mg. Percutaneous renal biopsy revealed a uniformly thickened glomerular and rigid basement membrane with immunoglobulin G (IgG) and complement C3 deposited along the glomerular capillary wall. Withdrawal of tiopronin-induced proteinuria complete remission and clinical resolution of nephrotic syndrome. Conclusions: Potential risk of kidney injury exists with long-term tiopronin treatment. Membranous nephropathy was a common renal pathologic feature. Proteinuria in the nephrotic range may spontaneously remit after tiopronin withdrawal. Periodic urine analysis and patient follow-up are recommended with tiopronin therapy.     

Long-term outcome of patients with membranous nephropathy after complete remission of proteinuria

To assess the prognostic significance of complete remission in patients with idiopathic membranous nephropathy, 33 patients were followed for a median of 96 months after remission of proteinuria. All patients had had a histological diagnosis of membranous nephropathy and a nephrotic syndrome. Only patients with a complete remission lasting for at least six months and with a follow-up of at least four years after remission were considered. No relapse of proteinuria developed in 17 patients (51%), 7 patients had relapse of non-nephrotic proteinuria (21%) and 9 (27%) relapse of nephrotic proteinuria. However proteinuria disappeared again in some patients so that at follow-up 73% of patients are in complete remission, 21% have non-nephrotic proteinuria and only 6% have nephrotic syndrome. All patients maintained a normal plasma creatinine over the years. It is concluded that complete remission of proteinuria is a strong predictor of long-term favourable outcome in patients with idiopathic membranous nephropathy.     

特发性与继发性膜性肾病的临床与病理分析

目的了解肾小球膜性病变的病因及临床、病理特点。方法分析统计我院肾脏病研究所经肾活检确诊的肾小球膜性病变189例。结果①特发性膜性肾病占28．57％，继发性膜性肾病占71．43%；②特发性膜性肾病平均年龄（46．24±15．31）岁，男女比例1.46：1。临床主要表现为蛋白尿、肾病综合征、高血压。肾病综合征的发生率为45．51％；③继发性膜性肾病最常见的病因依次为系统性红斑狼疮（70．37%）、乙型肝炎（28．89％）、银屑病（0．74％）。结论我院。肾小球膜性病变以继发性膜性肾病为主；特发性膜性肾病以男性、中年多见，大多为。肾病综合征；继发性膜性肾病最常见病因为系统性红斑狼疮、乙型肝炎和银屑病，病理分期主要为Ⅰ～Ⅱ级，病理表现中系膜细胞和基质增生在特发性膜性肾病和乙型肝炎病毒相关性肾炎、狼疮性肾炎之间差异显著。