IgA nephropathy in adults: immunohistologic findings and clinical course

Laboratory examination of specimens from 123 consecutive renal biopsies performed at Victoria General Hospital, Halifax revealed six cases of mesangial deposition, predominantly of IgA, unassociated with systemic disorders. Immunohistologic examination showed deposits of only IgA in one specimen, IgA and IgG in two and IgA, IgG and IgM in three. Glomerular deposits of C3 were seen in five of the specimens, and properdin was seen in three. Glomeruli in all the specimens showed increased matrix and increased numbers of cells in the mesangium. Electron microscopy revealed deposits in the mesangium or capillary wall in all five of the specimens so studied. All six patients had proteinuria, four had microscopic hematuria, and three had hypertension; in one patient the disease progressed to renal failure.     

运动致急性肾衰竭3例临床病理分析

目的:总结运动致急性肾衰竭(EIARF)病例的临床病理特点。方法:回顾分析本科2007年收治的3例EIARF患者的临床和肾脏病理资料。结果:EIARF临床表现为厌氧运动后恶心呕吐、腹痛、腰痛,血肌酐、尿酸升高,尿渗透压降低,伴或不伴肌酸激酶和肌红蛋白升高。肾脏病理1例表现为急性间质性肾炎伴局灶增生性肾小球肾炎,1例为IgA肾病(系膜增生性肾小球肾炎)。结论:EIARF少见,早期易误诊,临床应予重视。     

慢性肾功能不全患者的病理类型分析

目的分析肾活检时表现为慢性肾功能不全患者（Scr≥130μmol/L）的患者病理类型及特点。方法选择我院2006年1月～2008年12月行肾脏活检时表现为血清肌酐升高（Scr≥130μmol/L）的所有慢性肾脏病患者共104例作为研究对象,分析其临床和病理特点。同时重点分析了增生硬化型IgA肾病的特点。结果 104例慢性肾脏病患者在肾活检时表现为肾功能异常,占同期肾活检患者的11.9%。这些患者的平均血清肌酐（235.11±114.4）μmol/L。临床诊断主要为：慢性肾小球肾炎63例,肾病综合征27例,系统性血管炎4例,系统性红斑狼疮3例。其中78例（75.0%）患者有高血压。病理类型主要表现为IgA肾病（46.2%,其中有70.8%的患者主要表现为增生硬化性肾炎）,其次是间质性肾炎,系膜增生性肾小球肾炎,新月体肾炎,局灶节段肾小球硬化,IV型狼疮性肾炎。其中,增生硬化型的IgA肾病患者血压升高更为突出,但是尿蛋白量更少。结论表现为慢性肾功能不全的患者病理上主要为IgA肾病,其中增生硬化性肾炎是主要病理表现,其次是肾小管间质病变。慢性病变是主要的病理特点。     

表现为肾病综合征的IgM肾病及非IgA系膜增殖性肾炎的临床病理差异

分析临床表现为原发性肾病综合征的微小病变肾病（ＭＣＤ）、ＩｇＭ肾病（ＩｇＭＮ）和非ＩｇＡ、ＩｇＭ系膜增殖性肾炎（ＭｓＰＧＮ）病理改变（微机定量图像分析）和临床表现的联系。发现：（１）３种疾病以ＩｇＭＮ发生率最高（４８．８９％），一过性肾功损害、激素依赖比例以ＭｓＰＧＮ为高，ＩｇＭＮ、ＭｓＰＧＮ存在明显血尿；（２）病理定量分析表明：ＩｇＭＮ和ＭｓＰＧＮ的系膜细胞数、系膜基质增宽及间质改变较ＭＣＤ明显增高（Ｐ＜０．０１），ＭｓＰＧＮ系膜区增宽比ＩｇＭＮ更明显（Ｐ＜０．０１）；（３）ＩｇＭＮ、ＭｓＰＧＮ间质病变越重，越易伴有高血压、血尿、一过性肾功损害和激素依赖，其中以ＭｓＰＧＮ更明显。表明ＩｇＭＮ肾病预后相对较好，而ＭｓＰＧＮ相对较差。     

小儿肾小球疾病肾活检110例临床分析

目的探讨小儿肾小球疾病的病理特点及其与临床表现的关系.方法对110例符合肾小球疾病诊断标准的小儿行肾活检术,肾组织送光镜、电镜及免疫荧光检查.结果 110例肾组织病理改变为:IgA肾病32例(29.1%);系膜增生21例(19.1%);薄基底膜肾病18例(16.4%);膜增生性肾炎、Alport综合征及微小病变各6例(5.5%);局灶增生和轻微病变各4例(3.6%);新月体肾炎及增生硬化性肾炎各3例(2.7%);局灶节段性硬化、膜性肾病和IgM肾病各2例(1.8%);毛细血管内皮增生1例(0.9%).结论相同的临床诊断,其病理表现可不同;相同的病理诊断,也可有不同的临床表现.     

Renal vein thrombosis in nephrotic syndrome--a prospective study and review.

The incidence of renal vein thrombosis (RVT) and other thrombo-embolic phenomena was evaluated in 44 unselected patients with nephrotic syndrome. Renal vein thrombosis was demonstrated by selective renal venography in 10 patients and at post-mortem in one. Extension of the thrombus from the renal veins into the inferior vena cava was seen in 3 patients. Evidence of thrombo-embolism elsewhere in the body was seen in the form of thrombophlebitis in the lower extremities in 4 patients (9.1%), pulmonary embolism in 3 (6.8%) and myocardial infarction in one (2.3%). Of the 11 patients with RVT, renal histology showed membranous glomerulonephritis in 3, minimal change nephritis in 5, membrano-proliferative in one and focal and diffuse proliferative glomerulonephritis in one patient each. The characteristics clinical findings such as gross haematuria and flank pain were noted in only 3 patients with RVT. No significant difference could be detected between the plasma fibrinogen, serum cholesterol, beta-lipoprotein, triglycerides and phospholipid concentration of those who showed RVT and the remainder in whom RVT was not demonstrated. The possible mechanisms involved in the pathogenesis of RVT in nephrotic syndrome are discussed.     

复发肾病综合征重复活检肾脏组织学改变与糖皮质激素疗效的关系

目的了解复发肾病综合征患者重复肾活检其肾脏组织学变化特点及其对糖皮质激素疗效的影响。方法回顾性收集21例曾经标准糖皮质激素治疗完全缓解后复发并行重复肾活检患者的病理和临床资料,分析前后肾脏组织学变化与糖皮质激素疗效之间的关系。结果21例患者首次肾脏病理有IgA肾病10例,系膜增生性肾小球肾炎10例,膜增生性肾小球肾炎1例。共有9例患者肾脏组织学出现明显变化。重复活检有4例IgA肾病和1例系膜增生性肾小球肾炎病理亚型发生改变并伴有肾小管间质损害TIL分级改变,3例IgA肾病和1例膜增生性肾小球肾炎出现TIL分级改变,12例无明显组织学改变。5例重复活检病理亚型改变的患者均出现糖皮质激素抵抗,无改变者1例。结论肾病综合征复发后应尽早重复肾活检,IgA肾病易发生肾脏组织学改变,易出现难治性肾病综合征和糖皮质激素抵抗。     

Late development of purpura in a patient with glomerulonephritis

A 20-year-old man with a 10-year history of glomerulonephritis presented with a purpuric rash on his legs. A renal biopsy specimen obtained when he was 11 years old had shown mesangial glomerulonephritis; staining 9 years later for IgA had negative results. A second renal biopsy, performed when the rash was present, revealed mesangial glomerulonephritis and mesangial deposits of IgA; biopsies of the involved skin showed leukocytoclastic vasculitis. In this case isolated glomerulonephritis appeared to change to a multisystem illness, with a different immunologic character, through one of several possible pathogenetic mechanisms.     

IgA NEPHROPATHY

The clinical and pathological features of 54 Chi- nese patients with IgA nephropathy are reviewed. The diagnosis of IgA nephropathy is based on im munofluorescent staining that shows predominant mesangial IgA deposition in the absence of systemic diseases. The results indicate that in Chinese IgA nephropathy is a disease of young adults. The peak incidence is in the 20 t0 29 years old group. Pro. teinuria is the most common mode of clinical presen tation which is asymptomatic in 23 patients (42.6To) and in the nephrotic range in 7 patients (13%). Microscopic hematuria is also frequently found but only 9 patients (16.7%) present with gross hematuria. In addition, 4 patients are in renal failure at the time of diagnosis. The histopathology of IgA nephropathy is not essentially different from other idiopathic forms of glomerulonephritis. The glomerular lesions show features of focal proliferative change (50%), diffuse proliferative change (20.4%), focal or diffuse sclerosis (16.7%) and minimal glomerular lesions (13%). Membranous and membranoproliferative glomerular lesions'are not encountered. With im munofluorescenee, all cases show diffuse mesangial staining of IgA. With electron nucroscopy, the most characteristic feature is the presence of electron dense deposits in the glomerular mesangium. Three types of mesangial cells are identified and peripheral glo. merular capillary wall abnormalities are seen in 30% of the cases.     

攻膜复合物与肾小球肾炎

作者用免疫荧光法观察了攻膜复合物(Membrane Attack Complex,MAC)及IgG、IgA、IgM、C_3在正常及40例各种病理类型肾小球肾炎的肾组织中的分布。正常肾小球内仅在系膜区发现少量的MAC的沉积,但是肾小球肾炎的肾组织中,随着病理改变的加重,MAC的沉积增加,从单纯沉积于系膜区扩大到毛细血管壁,甚至在包曼氏囊及肾小球基底膜中也可发现。我们还注意到随着MAC在肾小球内沉积的增多、尿中蛋白量高,同时基底膜上阴离子电荷消失增多,提示肾小球肾炎肾组织的损伤与MAC直接相关。     

伴有系膜增殖的肾小球肾炎195例病理损伤分析

用光镜、免疫荧光显微镜及电镜观察了伴有系膜增生肾小球肾炎１９５例的病理损伤。结果表明，系膜增殖的程度与肾小球节段性硬化、新月体形成、毛细血管丛与球囊壁粘连、肾小管萎缩及肾间质纤维化密切相关。系膜增生可合并电子致密物内皮下沉积及基底膜变薄、撕裂等改变。系膜细胞增生亦是少数膜性肾炎的非特征性病变     

纤维样肾小球病1例报告并文献复习

目的结合文献,探讨纤维样肾小球病的诊断、治疗及预后。方法对我院肾病中心经肾活检确诊的1例纤维样肾小球病患者的临床资料,结合国内外相关文献进行分析。结果该例纤维样肾小球病患者为青年男性,l艋床表现为大量蛋白尿、低蛋白血症、高血压、肾功能异常,肾脏病理光镜表现为膜增殖性肾炎,免疫荧光IgG、IgA、IgM和补体均阳性,电镜下可见〈20nm的纤维丝沉积于肾小球系膜区。给予血管紧张索转化酶抑制制（ACEI）治疗后患者肌酐上升超过50％,给予糖皮质激索治疗2个月无效,且出现严重不良反应,肾衰竭加重。结论纤维样肾小球病是一种罕见的肾小球疾病,部分患者可找到病因,需依靠电镜诊断,目前缺乏有效的治疗方法,该病预后差,大多数患者会进入终末期肾衰。     

儿童肾小球疾病临床病理研究

为了探讨肾小球疾病的病理类型与临床治疗的关系，对肾小球疾病１３１例行肾穿刺活组织检查。其中原发性肾小球疾病１２０例，继发性肾小球疾病１１例，前者以系膜增生性肾小球肾炎（ＭｓＰＧＮ）居多，占２９．２％，其次是ＩｇＡ肾病（ＩｇＡＮ），占２５．８％。临床诊断与病理诊断符合率为５７．２％。治疗后，随访６个月至６年，总痊愈率和好转率为７６．７％，病理类型中的微小病变（ＭＣＮＳ）、轻度ＭｓＰＧＮ、ＩｇＭ肾病（ＩｇＭＮ）、毛细血管内增生性肾小球肾炎（ＥｎＰＧＮ）等的疗效及预后好，膜增生性肾小球肾炎、重度ＭｓＰＧＮ及组织损害严重的ＩｇＡＮ等疗效差。提示治疗效果、预后与病理类型密切相关     

成人无症状性血尿蛋白尿的临床病理分析及预后

目的：探讨成人无症状性肾小球肾炎的临床与病理关系，了解其早期肾功能的变化。方法：54例临床诊断为隐匿性肾炎的患者均行肾组织活检术，肾组织行光镜，免疫组化及电镜检查，同时查肝肾功，血脂，尿蛋白定量，肾功效免，自身抗体，乙肝系列等。结果：单纯性血尿30例占55．5％；余为单纯性蛋白尿11例及血尿，蛋白尿13例，肾脏病理改变以轻度系膜增生性肾炎（MsPGN24例）及IgA肾病(IgAN15例）为主，分别占44．4％，27．7％，余为局灶增生性肾炎（FPGN），局灶段肾小球硬化症（FSGS），IgM肾病，Ⅰ型膜增生性肾炎（MPGN），毛细血管内增生性肾炎（EnPGN),51.8%伴小血管炎（28例）。结合临床可见：发作性肉眼血尿多见于IgAN,单纯性蛋白尿多见于MsPGN,早期肾功损害主要见于IgAN,FSGS,28例随访6月至5年，仅2例血Scr轻度升高。结论；成人隐匿性肾炎临床以单纯血尿和／或蛋白尿为主要表现，其病理类型多样，但以MsPGN及IgAN为主，发作性肉眼血尿多为IgAN,单纯性蛋白尿多见于MsPGN,约51．8％伴明显小血管炎，约40％有早期肾功能损害，对于发作性肉眼血尿或单纯性血尿，蛋白尿，肾活检有重要价值。     

IgA型感染后肾小球肾炎的临床病理与预后研究

目的观察IgA型感染后肾小球肾炎(PIGN)的临床与病理特征,及其与金黄色葡萄球菌感染及潜在糖尿病的相关性。方法回顾性分析IgA型PIGN患者25例,均进行肾组织活检,分析其在光镜病理、免疫荧光显微镜和电子显微镜下变化特点,总结其临床结局。结果所有患者在发病时均为临床金黄色葡萄球菌感染其中MSSA感染11例,MRSA感染14例。肾活检显示:急性期PIGN 6例,表现为弥漫性增生性肾小球肾炎,恢复期或亚急性期显示系膜细胞增生。免疫荧光显示IgA沉积。超微结构分析表明局灶性上皮下驼峰状电子致密物沉积。25例均使用抗生素治疗12例联合应用甲泼尼龙治疗,血液透析依赖8例。其中16例出院,完全缓解6例,部分缓解10例;9例持续住院,死亡5例。结论 IgA型与金黄色葡萄球菌感染有关。诊断主要依靠IgA的免疫荧光染色和存在毛细血管内增殖,电镜检查示上皮下电子致密沉积。     

原发性肾小球疾病97例临床病理分析

目的: 探讨原发性肾小球疾病患者临床表现与病理之间的关系、肾活检的并发症及其临床意义。方法: 分析97例原发性肾小球疾病患者的临床表现、病理类型及肾活检资料。结果: 97例原发性肾小球疾病中,IgA肾病30例,系膜增生性肾小球肾炎26例,膜性肾病10例,局灶性节段性硬化性肾小球肾炎14例,IgM肾病2例,毛细血管内增生性肾小球肾炎1例,肾小球轻微病变8例,增生硬化性肾小球肾炎4例,新月体性肾小球肾炎2例。肾活检术后并发症:肉眼血尿3例,肾包膜下小血肿2例,无其它严重并发症。结论: 肾活检病理检查与临床密切结合,有助于原发性肾脏疾病正确诊断和治疗。肾穿刺术虽有一定并发症,但多不严重,是一项安全、简便的诊断操作。     

NON-IgA MESANGIAL PROLIFERATIVE GLOMERULONEPHRITIS CLINICAL AND PATHOLOGICAL ANALYSIS OF 77 CASES

Primary mesangial proliferative glomerulonephritis without IgA deposition (non-IgA MsPGN)is one of the most common types of glomerular disease in China .In an attempt to investigate its clinical and pathological features ,we reviewed 77 such cases from 380 patients with primary glomerulonephritis taken renal biopsies during 1980-1987. Prodromal upper respiratoty tract infection occurred in 31 cases (40%) .In immunofluorescence Microscopy .prominent IgG granular deposits in mesangium were observed in 45 cases (58%) .These features are quite different from those in western countries ,indicating it might have different pathogenetic ptocesses. According to the severity of mesangial lesions , the 77cases were divided into 3 groups: mild (55 cases ) ,moderate (14) and severe (8) .In the patients with mild mesangial lesion and massive proteinuria , the therapeutic response to prednisone was similar to that in adult minimal change disease . In the moderate and severe groups ,there was a significantly higher incidence of superimposed tubulo – interstitial lesions associated with hypertension, persistant renal insufficiency and a poor response to prednisone . This work showed non-IgA MsPGN covered about 20% of our primary glomerulopathy ,which may be related to a higher incidence of infection. It was suggested that minimal change nephritic syndrome ,inspite of the variety of immunoglobulin mesangium deposits, could be treated as a single disease entity, and light microscopy is most important in offering prognostic information.     

Glomerulonephritis with mesangial deposits of IgA unassociated with systemic disease.

Typical features of IgA-associated nephritis were found in renal biopsies from 16 of 355 consecutive patients. Generalized segmental mesangial proliferation was noted in biopsies from most patients, and dense deposits were detected by electron microscopy in mesangial regions of approximately 50% of biopsies. Immunofluorescent studies showed IgA to be the predominant immunoglobulin in glomueruli; IgG was present in less than 50% of biopsies and IgM in only 12%. The serum IgA value was significantly increased (P les than 0.001) in 50% of patients and the mean IgA/IgG ratio was significantly increase (P less than 0.001) for the patient group as a whole, which suggests a selective increase in IgA. Mesangial deposits of C3 were present in 15 of 16 biopsies and properdin was noted in all biopsies tested; C4 was not demonstrated in any biopsy. This suggests activation of the alternative complement pathway. The results of this study support the concept that IgA-associated nephritis is a unique condition that in some patients gives rise to idiopathic recurrent hematuria. Although the prognosis is good in the majority of patients, the renal disease may progress.     

水痘病毒感染伴肾小球肾炎和脑炎1例报告

患者15岁,男性,因水痘、肾炎和肾病综合征、肾功能损伤、补体C3下降2个月而入北京中日友好医院肾内科治疗,肾活检证实为毛细血管内增生性肾小球肾炎伴足细胞增生和肾小管损伤,直接免疫荧光显示IgG、IgA、IgM、C3、C1q和纤维蛋白原沿肾小球毛细血管壁和系膜区颗粒状和团块状沉积,透射电子显微镜检查显示电子致密物在肾小球多部位沉积,血清中抗水痘病毒抗体(IgM)阳性,免疫组织化学和原位杂交显示水痘病毒抗原和mRNA存在于肾小球和肾小管上皮细胞,特殊染色和透射电子显微镜显示病毒颗粒和病毒包涵体存在于肾小球和肾小管上皮细胞内。患者住院期间尚有癫痫发作,脑电图及核磁共振证实为病毒性脑炎导致的癫痫,故确诊为水痘病毒感染伴发肾小球肾炎和脑炎。这是1例通过分子病理学方法证实水痘病毒在肾内感染导致的肾小球肾炎。     

158例肾活检病理及临床分析

目的回顾性总结158例肾活检的病理结果,分析其病理类型及临床分类特点。方法总结我院158例肾活检病理资料,分析其病理类型与临床分类特点。结果158例肾活检中原发性肾小球疾病125例(79.11%),继发性肾小球疾病33例(20.89%)。原发性肾小球疾病病理类型最多的是系膜增生型肾炎,第二位是IgA肾病,继发性肾小球疾病以紫癜性肾炎病理类型最多。原发性肾小球疾病临床分布最多的前三位依次是肾病综合征、慢性肾炎、IgA肾病。其中肾病综合征的病理类型主要是系膜增生型和膜性肾病,慢性肾炎的主要病理类型是系膜增生型和肾小球硬化。结论原发性肾小球疾病是最常见的肾小球疾病,肾活检病理类型以系膜增生型肾炎、IgA肾病最多,临床类型则以肾病综合征、慢性肾炎、IgA肾病最为多见。