237例肾组织活检的临床病理分析

目的:旨在通过回顾分析237例肾活检的病理类型与临床诊断,对两者的相关性进行研究总结.方法:分析237例行肾活检病人的临床诊断、免疫病理、光镜、电镜的改变,做出病理诊断.结果:原发性肾小球疾病占78.1％,主是IgA肾病、系膜增生性肾小球肾炎、膜性肾病、微小病变等.继发性肾小球肾炎占19.8％,主是狼疮性肾炎、紫疲性肾炎等.发病年龄平均为37.12岁.讨论:原发性肾病是较常见的类型,以IgA肾病为主,继发性肾病则以狼疮性肾炎为主;绝大多数的慢性肾炎综合征患者的病理表现呈现出多样化的特征,这使得肾活检意义重大.     

肾病综合征1009例病理类型及流行病学分析

目的探讨肾活组织病理检查（下称肾活检）在肾病综合征诊治中的意义。方法选择近10年诊断为肾病综合征患者1009例，分析肾活组织穿刺检查术后的病理结果。结果原发性肾小球肾炎占71．9％，病理类型以非IgA系膜增生性肾炎、IgA肾病及膜性肾病多见；继发性肾病综合征占28．1％，其中居前3位的是狼疮性肾炎、过敏性紫癜性肾炎和乙肝病毒相关性肾炎。结论肾活检对肾病综合征的诊断、治疗及评估预后有重要意义。     

86例膜性肾病回顾性分析及对CD4^＋CD^25＋调节性T细胞的初步研究

目的：回顾性总结、分析86例膜性肾病患者的临床表现、实验室检查及肾活检病理的特点及相互联系,认识膜性肾病的发病和流行病学特点。通过对特发性膜性肾病（idiopathic membranous nephropathy,IMN）患者和正常人外周血CD4＋CD2＋5调节性T细胞（Treg细胞）数量的检测,了解Treg在IMN患者外周血的变化规律,探讨其在IMN发病中的作用。方法：2004年3月～2008年12月间病理确诊为膜性肾病患者86例,分析患者一般资料、病理类型和临床特征。选择2007年～2008年IMN患者10例,随机选取与IMN患者年龄相匹配的健康志愿者10例,检测所有对象外周血Treg细胞数量。结果：（1）86例膜性肾病患者,其中IMN68例,占80%,4例患者随访后确诊为恶性肿瘤;继发性膜性肾病18例（其中乙肝病毒相关性肾炎5例,狼疮性肾炎4例,移植肾肾小球肾炎1例）,占20%。（2）IMN免疫荧光以IgG沉积为主,乙肝病毒相关性肾炎C1q沉积较IMN多（P〈0.05）,并均存在HBsAg沉积,与IMN相比狼疮性肾炎C1q沉积明显增多,C4也多于IMN（P〈0.05）。（3）病理分期分布特点：Ⅱ期膜性肾病多见。（4）IMN患者治疗前外周血Treg细胞占CD4＋淋巴细胞的百分比为（7.46±0.94）%,正常对照组为（6.54±1.0）%。结论：（1）根据病因分为IMN及继发性膜性肾病两种,男性发病率大于女性,中老年多发,继发性膜性肾病的年龄及性别分布根据病因的不同而有所不同,临床表现均以肾病综合征表现为主;IMN发病率明显大于继发性膜性肾病。（2）免疫荧光检查：IMN以IgG及C3沉积为主,乙肝病毒相关性肾炎均存在乙肝表面抗原,狼疮性肾炎与乙肝病毒相关性肾炎的C1q沉积较特发性膜性肾病明显增多（P〈0.05）。（3）IMN患者外周血Treg细胞数量较正常人增多。     

原发性干燥综合征合并膜性肾病

目的分析原发性干燥综合征（pSS）患者合并膜性肾病（MN）的临床和肾脏病理特点，以提高对此类疾病的认识。方法回顾性分析北京协和医院2001年5月至2006年5月期间17例原发性干燥综合征伴膜性肾病的临床和肾脏病理特点。结果17例患者中男性6例，女性11例，平均年龄（47．3±17．3）岁（24～72岁）。主要临床表现为水肿和蛋白尿（17/17），其中肾病综合征占13／17，镜下血尿占12／17。根据光镜、免疫荧光和电镜的特点，17例患者的肾脏病理可分为单纯MN组（8／17）和不典型MN组（9／17）。单纯MN组：光镜下未见系膜细胞和基质增生；电镜下电子致密物（ED）仅在上皮下和（或）肾小球基底膜内沉积。不典型MN组：光镜下可见系膜细胞和基质节段或弥漫增生；免疫荧光除了IgG阳性外，常有早期补体成分C1q阳性（7／9）；电镜下可见ED分布于上皮下和系膜区。17例患者中有2例患者接受了重复肾活检，其中1例首次活检表现为MNI期，8年后再次活检表现为不典型MN；另1例首次活检表现为不典型MN，4年后再次活检却表现为狼疮肾炎（LN）Ⅳ-G（A／C）＋V型。结论原发性干燥综合征合并膜性肾病并非少见，是干燥综合征合并蛋白尿常见的病因。对于光镜下呈现不典型膜性肾病和免疫荧光早期补体成分阳性，电镜下有系膜区ED沉积的患者应警惕若干年后转化为隐匿性狼疮肾炎的可能性。     

几种重要肾小球肾炎的免疫组化诊断与鉴别诊断及其进展

肾活检病理诊断的特殊性在于它由光镜、免疫病理及超微病理三部分组成.三种手段相辅相成,有时缺一不可.免疫荧光或免疫组化技术对肾脏疾病的诊断及其分类具有重要作用,因而在肾活检病理诊断中占有极为重要的地位.许多肾病的诊断通常需要证实有无免疫球蛋白、轻链或补体成份的特征性沉积,如膜性肾病、IgA肾病、抗肾小球基膜肾炎、轻链肾病以及定义不明确或有争议的疾病如免疫触须样肾小球肾炎、IgM肾病和C1q肾病等.     

合并肾损伤的乙肝患者的临床及病理分析

目的:探讨乙肝合并原发性肾病与乙肝相关性肾炎(HBV-GN)的临床及病理差异。方法:选择2005年01月—2019年12月在联勤保障部队第九六〇医院住院、血清HBsAg阳性合并肾损伤患者74例。根据肾脏病理诊断进行分组，A组为乙肝相关性肾炎患者24例，B组为乙肝合并原发性肾病患者50例。比较两组患者的临床及病理差异特点。结果:(1)临床指标中，两组患者首发症状、入院诊断、谷草转氨酶、乙肝病毒DNA定量、血红蛋白、镜下血尿、补体水平差异有统计学意义(P<0.05),两组患者性别、谷丙转氨酶、血肌酐、白蛋白、24 h尿蛋白水平差异无统计学意义(P>0.05);(2)病理指标中，两组患者肾脏病理类型、IgG、C3、C1q和FRA沉积差异有统计学意义(P<0.05),两组患者肾脏病理特点、IgA和IgM沉积差异无统计学意义(P>0.05)。结论:乙肝相关性肾炎患者临床表现以肾病综合征多见，病理类型多见于膜性肾病，并且更易出现临床症状(肝损伤、贫血、补体下降等)及肾脏免疫复合物沉积。乙肝合并原发性肾病患者临床以肾病综合征和慢性肾炎多见病理改变以膜性肾病和系膜增生性病变为主...     

1148例新疆地区肾活检病理资料分析

目的:分析肾活检资料的地域、种族特点,为肾脏疾病的特异性诊治提供依据.方法:回顾性分析新疆医科大学第一附属医院1 148例肾活检病例的临床资料,进行统计学分析.结果:原发性肾小球肾炎占76.1%,其常见病理类型为非IgA肾病、IgA肾病;继发性肾小球肾炎占13.0%,其中以狼疮性肾炎最为常见;各病理类型分布在维吾尔族与...     

996例肾活检临床病理分析

目的 回顾性分析本院肾脏内科近10年肾活检病理类型及疾病谱变化.方法 收集2006年2月至2016年2月期间996例肾活检患者临床病理资料,分析肾脏疾病临床病理特点.结果 996例肾活检患者中,原发性肾小疾病760例,占76.31％,继发性肾小球疾病211例,占21.18％,肾小管间质性疾病22例,占2.21％,遗传性肾病3例,占0.30％.原发性肾小球疾病最常见病理类型为IgA肾病238例,占31.32％,其次为系膜增生性肾炎199例,占26.18％,膜性肾病127例,占16.71％.继发性肾小球疾病最常见为狼疮性肾炎76例,占36.02％,其次为紫癜性肾炎56例,占26.54％,乙肝相关性肾炎24例,占11.37％.原发性肾小球疾病临床表现最常见为肾病综合征446例,占58.68％,其次为慢性肾炎223例,占29.34％,隐匿性肾小球肾炎69例,占9.08％.结论 原发性肾小球疾病是目前临床最常见肾小球疾病,其病理类型以IgA肾病最为常见,近年来膜性肾病所占比例明显增高.继发性肾小球疾病以狼疮性肾炎最为常见,乙肝相关性肾炎所占比例较前增高.     

IgA肾病研究新进展

IgA肾病是最常见的原发性肾小球性肾炎,最新定义为自体肾活检组织免疫荧光或免疫酶标染色显示以IgA或IgA沉积为主的肾小球疾病（不要求所有肾小球均有IgA沉积）,同时除外狼疮性肾炎等继发性IgA沉积。IgA沉积须有一定强度,分布于系膜区,可伴有或不伴有毛细血管袢沉积。除外单纯膜性、弥漫、球性、颗粒状或线状肾小球基底膜（GBM）沉积。     

狼疮肾炎患者肾组织免疫物质沉积与临床关系探讨

目的：分析狼疮肾炎（lupusnephritis,LN）患者肾脏免疫物质沉积强度与病理分型及临床指标的关系。方法：回顾性分析41例狼疮性肾炎患者（均行肾活检）的临床表现、实验室指标和病理资料。结果：41例LN患者均有蛋白尿,临床分型以肾病综合征型最为常见（20/41,48.7%）,其次为肾功能不全型12例（29.2%）,肾炎综合征型7例（17%）,隐匿性肾炎型2例（4.8%）。各免疫物质IgA,IgG,IgM,C3,C4,C1q）基本呈＂满堂亮＂沉积,以Ⅴ型（合并Ⅳ型、Ⅲ型）LN的IgG沉积明显,但差异无统计学意义。抗ds-DNA滴度与肾脏穿刺组织中C3的沉积强度呈正相关关系（P〈0.05）;但SLEDAI评分、24h尿蛋白定量、血肌酐与肾脏免疫物质沉积未见明显相关性。结论：LN患者的临床表现与肾脏病理改变复杂多样,两者有一定的相关性;不同病理类型的LN,其发病机制可能存在差异;肾组织补体C3荧光沉积强度可以作为病情严重及活动的参考指标。     

Glomerular deposition of mannose-binding lectin in human glomerulonephritis.

BACKGROUND: Mannose-binding lectin (MBL), a member of the collectin family, binds to various oligosaccharides and activates the classical pathway of complement independent from C1q. At present it is unknown whether this so-called lectin pathway of complement activation plays a role in the pathogenesis of human glomerulonephritis. METHODS: Direct immunofluorescence of 84 renal biopsies using an MBL-specific monoclonal antibody and antibodies directed against IgG, IgA, IgM, C1q, C3, and terminal complement complex (TCC) was performed. Serum MBL levels of 50 patients were determined by enzyme-linked immunosorbent assay. RESULTS: MBL was detected in the glomeruli of patients with lupus nephropathy (15 of 16), membranous nephropathy (10/15), membranoproliferative glomerulonephritis type I (5/6) and anti-GBM nephritis (2/4). MBL deposition paralleled that of immunoglobulins, C1q, C3, and TCC but was less intense as compared to C1q. Focal segmental deposits of MBL were present in focal segmental glomerulosclerosis (4/6), IgA nephropathy (3/11), amyloidosis AL (1/4), and advanced renal fibrosis (2/2). Here MBL staining was identical to IgM and C3 and considered an unspecific entrapment of MBL in sclerotic lesions in these cases. No significant difference in MBL serum levels was observed between normal controls and patients with lupus nephritis, membranous nephropathy, membranoproliferative glomerulonephritis, focal segmental sclerosis, minimal change disease or IgA nephropathy. In patients suffering from membranous nephropathy with (n=10) or without (n=5) glomerular MBL deposits serum creatinine, C3, C4, serum protein, and proteinuria were not statistically different. CONCLUSION: MBL is present in the glomeruli of patients with glomerulonephritis involving deposition of IgG and activation of the classical pathway of complement. We propose that MBL binds to agalactosyl oligosaccharides of IgG that terminate in N-acetylglucosamine. The extent to which the lectin pathway of complement contributes to overall complement activation in the glomeruli remains unknown, but is likely to be marginal.     

High prevalence of anti-C1q antibodies in biopsy-proven active lupus nephritis.

BACKGROUND: Anti-C1q antibodies (anti-C1q) have been shown to correlate positively with systemic lupus erythematosus (SLE) nephritis. Several clinical studies indicated a high negative predictive value, suggesting that active lupus nephritis is rarely seen in patients with no anti-C1q. However, the true prevalence of anti-C1q at the time of active lupus nephritis has not been well established. The aim of this study was to determine prospectively the prevalence of anti-C1q in proven active lupus nephritis at the time of the renal biopsy. METHODS: In this prospective multi-centre study, we investigated adult SLE patients undergoing renal biopsy for suspected active lupus nephritis. Serum samples were taken at the time of the biopsy and analysed for the presence of anti-C1q in a standardized way. The activity of lupus nephritis was classified according to the renal histology. Biopsies were also analysed for the presence of glomerular IgG, C1q and C3 deposition. RESULTS: A total of 38 patients fulfilling at least 4/11 American College of Rheumatology (ACR) criteria for the diagnosis of SLE were included. Out of this, 36 patients had proliferative (class II, III or IV) and two had class V lupus nephritis. All but one patient with proliferative lupus nephritis were positive for anti-C1q (97.2%) compared with the 35% of control SLE patients with inactive lupus nephritis and 25% of SLE patients without lupus nephritis ever. All patients were positive for glomerular C1q (36/36) and 37/38 patients had glomerular IgG deposits. Anti-C1q strongly decreased during successful treatment. CONCLUSIONS: Anti-C1q have a very high prevalence in biopsy-proven active lupus nephritis, thus a negative test result almost excludes active nephritis. The data support the hypothesis of a pathogenic role of anti-C1q in lupus nephritis.     

Immunohistochemical detection of immunoglobulins and complements in formaldehyde-fixed and paraffin-embedded renal biopsy tissues; an adjunct for diagnosis of glomerulonephritis

BACKGROUND: The present study was undertaken to demonstrate the deposition of immunoglobulins or complements in formaldehyde-fixed and paraffin-embedded renal biopsy tissues through the unmasking of antigens with microwave treatment plus protease digestion or trypsin digestion. METHODS: Biopsy samples from patients with IgA nephritis (n = 7), lupus nephritis (7), membranous nephropathy (7) and mesangiocapillary glomerulonephritis (3) were used. Antigen unmasking was performed with (i) microwave treatment plus protease digestion for 10, 30 or 60 min, or (ii) digestion with 0.25% trypsin for 60 or 120 min. RESULTS: Microwave treatment plus protease digestion for 30 or 60 min and trypsin digestion for 120 min provided good results for the unmasking of immunoglobulins in glomeruli with structural preservation. The IgA deposits in IgA nephritis and IgG deposits in lupus nephritis and membranous nephropathy were clearly revealed in more than 80% of cases by both pretreatments. Microwave treatment plus protease digestion for 30 min revealed the deposition of C3 in all cases of mesangiocapillary glomerulonephritis and lupus nephritis and was superior to trypsin digestion. Characteristic patterns of C3 deposition were observed for these forms of glomerulonephritis, although C3 deposits in membranous nephropathy were detected in only 50% of cases. It was not possible to unmask all of the antigens in the glomeruli, especially those with weak immunofluorescence. CONCLUSION: Microwave treatment plus protease digestion is effective for the unmasking of antigens in paraffin sections and as useful for the diagnosis of immune-mediated glomerulonephritis as trypsin digestion.     

Recurrent membranous nephropathy and leiomyosarcoma in the renal allograft of a lupus patient

The frequency of membranous lupus nephritis recurrence (World Health Organization (WHO) class V) in the allograft after renal transplantation is unknown, but it appears uncommon (only two reported cases in the literature). Despite the increased incidence of sarcomas in organ transplant recipients (compared to the general population), non-Kaposi's sarcoma is an uncommon malignancy, and primary tumor involvement of a renal allograft is a rare occurrence. Our patient is a 28 year old female with end-stage renal disease (ESRD) secondary to membranous lupus nephritis who received a living related transplant from her mother. At 26 months post-transplant, she presented with proteinuria and a rise in creatinine (Cr). Allograft biopsy was consistent with recurrent membranous nephropathy. Five weeks later, she was found to have a high-grade leiomyosarcoma originating within the allograft. We reviewed the literature on recurrent post-transplant membranous nephropathy and the possible role of the Epstein-Barr virus (EBV) infection in smooth muscle tumors occurring in organ transplant recipients. We also considered the association of membranous nephropathy and malignancy.     

C1q nephropathy: a distinct pathologic entity usually causing nephrotic syndrome

The presence, distribution, and intensity of glomerular C1q localization were evaluated by direct immunofluorescence microscopy in 800 renal biopsy specimens which were also studied by light and electron microscopy. Identified were 15 patients with extensive (mean: 3.6 + out of 4 +), predominantly mesangial, C1q localization along with C3 and immunoglobulins, but no evidence for systemic lupus erythematosus. Pathologically, this lesion most closely resembled lupus nephritis. Clinical and pathologic data from these 15 C1q nephropathy patients were compared to data from 30 lupus nephritis and 223 other proliferative glomerulonephritis patients, and the C1q nephropathy patients were found to be dissimilar to both groups. The 15 C1q nephropathy patients had an average age of 17.8 years, 8 males, 7 females, 9 Black, 100% had proteinuria (mean 7.5 g/d), 40% hematuria, 0% hypocomplementemia, and 0% antinuclear antibodies. By electron microscopy, 100% had mesangial dense deposits, 20% capillary wall dense deposits, and 0% endothelial tubuloreticular inclusions. Nine patients treated with steroids had no definite resolution of proteinuria. We proposed that C1q nephropathy is a distinct clinicopathologic entity, usually causing steroid-resistant nephrotic syndrome in older children and young adults.     

Clinicopathological study of originally non-lupus “full-house” nephropathy

Background: Glomerular “full-house” immunofluorescence staining commonly indicates lupus nephritis. However, some non-lupus nephropathy also can present with a “full-house” immunofluorescence pattern mimicking lupus nephritis. The goal of this study is to define the clinicopathological spectrum of originally non-lupus “full-house” nephropathy. Methods: Records of 24 patients with “full-house” nephropathy in the absence of clinical or serological evidence of systemic lupus erythematosus (SLE) at the time of renal biopsy were abstracted for demographics, clinical presentation, laboratory data, renal biopsy findings, and clinical follow-up. Results: The clinicopathological diagnoses included membranous glomerulonephritis (GN) (46%), IgA nephropathy (21%), membranoproliferative GN (12.5%), postinfectious GN (12.5%), C1q nephropathy (4%), and unclassified mesangial GN (4%). No one had endothelial tubuloreticular inclusions. One patient originally diagnosed as IgA nephropathy developed anti-DNA antibody and another one patient with membranous GN developed hypocomplementemia 8 months and 10 months after renal biopsy, respectively. The two patients also developed clinical symptoms of lupus subsequently. Conclusions: There was a broad spectrum of glomerular histological findings in non-lupus “full-house” nephropathy. The possibility of “full-house” nephropathy preceding the emergence of overt systemic lupus erythematosus remained to be elucidated.     

Occurrence of anti-C1q antibodies in IgA nephropathy

Background: The pathogenic mechanisms and the antigens involved in the establishment and progress of IgA nephropathy are unknown. As antibodies against C1q have been reported to correlate with SLE nephritis, we analysed the occurrence of these antibodies in IgA nephropathy in order to investigate the possibility of pathogenetic similarities in these renal disorders. Methods: The occurrence of IgA- and IgG anti-C1q antibodies (anti-C1q) were determined by ELISA in patients with IgA nephropathy (n=36) and SLE nephritis (n=37), diseases both known to be associated with circulating immune complexes. Levels of these antibodies were also determined in two other glomerular diseases, i.e. idiopathic membranous glomerulo-nephritis (n=7) and minimal change disease (n=2), in which circulating immune complexes are usually not present, and in 40 healthy controls. Results: IgA anti-C1q was observed in increased titres in 11/36 of the patients with IgA nephropathy, in 2/37 of the patients with SLE nephritis (both with proliferative disease) and in 1/9 of the patients with membranous and minimal change disease (P<0.001). Increased titres of IgG anti-C1q were observed in 1/36 of the patients with IgA nephropathy, in 17/37 of the patients with SLE nephritis and in 0/9 of the patients with membranous and minimal change disease (P<0.001). There were no correlations between the levels of anti-C1q antibodies and clinical parameters such as degree of proteinuria, haematuria, or renal function. Nor was there any correlation to the concentration of C3a and the terminal complement complex (TCC) in patients with IgA nephropathy. Conclusions: The occurrence of anti-C1q antibodies in both IgA nephropathy and SLE nephritis, albeit of different predominating isotypes, indicates the possibility of a similar pathogenic mechanism involved in these renal disorders. The occurrence of IgA anti-C1q antibodies in patients with IgA nephropathy has to our knowledge not previously been reported.