肾脏有免疫复合物沉积的小血管炎的临床病理特点

目的 回顾性总结分析肾脏有免疫复合物沉积的ANCA相关系统性血管炎(AASV)患者的临床和病理特点。方法 将近5年在北京大学第一医院住院诊断为AASV且肾活检免疫荧光显示系膜区有免疫复合物沉积(免疫球蛋白≥++)的患者与同期少免疫复合物沉积的患者的临床和病理进行比较。结果 肾脏有免疫复合物沉积者8例(IgM沉积5例,IgA沉积2例,IgG沉积1例),肾脏少免疫复合物沉积者32例。2组在性别、年龄、ANCA类型、起病至肾活检的时间、临床表现以及短期肾脏存活率方面差异均无显著性意义,仅前组患者在有前驱感染方面显著多于后组(P<0.05)。结论 肾脏有免疫复合物沉积的AASV的临床与病理特点与普通的AASV基本一致,应引起高度重视,而需要针对AASV的强化免疫抑制治疗。     

ANCA相关性血管炎肾脏及其他系统损害的临床和病理与预后的相关研究

目的探讨抗中性粒细胞胞浆抗体相关性血管炎(antineutrophil cytoplasmic antibodyassociated vasculitis,AAV)肾损害及其他系统损害患者的临床和病理特征,并对其预后及其相关危险因素进行分析。方法选择1995年9月至2009年9月内蒙古自治区人民医院明确诊断为AVV内蒙古籍患者123例,血清抗中性粒细胞胞浆抗体(antineutrophil cytoplasmic antibody,ANCA)阳性,根据检测结果将患者分为胞质型(C-ANCA)阳性组和核周型(P-ANCA)阳性组,进行临床、实验室、病理及预后相关因素进行分析。随访终点为死亡或终末期肾衰竭进入规律肾脏替代治疗,平均随访时间5年。结果 123例患者中男63例,女60例,男女比例1.05:1,年龄38~82岁,平均年龄(60.0±22.0)岁,50~82岁患者102例(占82.9%)。其中P-ANCA阳性109例,C-ANCA阳性14例。P-ANCA组肾脏、肺脏受累与C-ANCA组相似,消化道、关节、眼损害发生率低于C-ANCA组(P0.05);耳、皮肤、肌肉、神经系统损害2组无统计学差异。123例患者中有62例患者行肾活检,其中细胞性新月体肾炎30例(占48.4%),毛细血管袢坏死12例(占19.4%),细胞伴纤维性新月体形成15例(占24.2%),纤维性新月体伴局灶节段性硬化5例(占8.1%)。对AAV患者进行5年随访,对患者死亡或进入肾脏终末期代替治疗进行相关因素分析:C-ANCA组与P-ANCA组相比,患者死亡与进入终末期替代治疗2组间均无统计学差异(P0.05)。对患者的年龄、性别、ANCA类型、发热、肾衰竭、呼吸衰竭、咯血、脏器受累数目、多脏器衰竭、肺感染因素进行COX回归分析:呼吸衰竭、多脏器衰竭为死亡的相关因素(偏回归分数分别为2.087,1.129,均P0.05)。对患者起病时实验室数据进行回归分析:血肌酐、红细胞沉降率、血浆白蛋白、血红蛋白与终末终点相关。Logistic回归分析,起病的血肌酐及红细胞沉降率是预测患者预后的独立危险因素。结论 AAV以中老年男性多见,PANCA阳性患者较C-ANCA阳性患者,发病率高,受累器官数目多且病程谱广。呼吸衰竭、多脏器衰竭与患者死亡密切相关。患者起病的血肌酐及红细胞沉降率是预测患者预后的独立危险因素。     

肾脏有免疫复合物沉积的抗肾小球基底膜病的临床病理特点

目的了解伴有免疫复合物沉积的抗肾小球基底膜(GBM)抗体相关疾病的肾脏病理表现及其临床特点.方法将我科近9年来确诊并行肾穿刺活检证实的10例伴有免疫复合物沉积的抗GBM抗体相关疾病患者与同期25例经典抗GBM肾炎患者的临床和病理资料进行比较.结果10例患者中,2例为膜性肾病,1例IgA肾病,1例膜增生性肾炎,1例过敏性紫癜性肾炎,1例膜型乙肝病毒相关性肾炎,其余4例系膜区有免疫复合物沉积,但原发疾病不清.与经典抗GBM肾炎患者比较,2组在年龄、性别、临床表现、血清学检查、肾脏病理、治疗及转归等方面无显著性差异.结论抗GBM肾炎可发生在其它肾小球疾病的基础上,其临床和病理特点与经典的抗GBM肾炎基本一致.临床上应及时检测血清抗GBM抗体,以利于早确诊而选择血浆置换和强化免疫抑制治疗.     

ANCA相关性小血管炎肾损害的治疗

韦格纳肉芽肿（WG）、显微镜下型多血管炎（MPA）、Chury-Strauss综合征（CSS）和寡免疫复合物沉积型坏死性新月体肾炎（NCGN）发病机制与ANCA相关,目前统称为抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎（ANCA-associated systemic vasculitis,AASV）。     

ANCA相关性小血管炎所致急性肾衰竭临床分析

原发性小血管炎伴或不伴抗中性粒细胞胞浆抗体（ANCA）阳性,临床上常出现多系统受损。我国环核型ANCA（pANCA）相关小血管炎较多见,肾脏损害严重,预后差。在肾活检没有普及的基层医院,其最主要的诊断依据是血清ANCA检测结果,误诊率较高。现将我院近8年收治的9例pANCA相关小血管炎所致急性肾衰竭患者的临床资料总结如下。     

丙基硫氧嘧啶致抗ANCA阳性小血管炎5例分析

目的：研究丙基硫氧嘧啶（PTU）引起的抗中性粒细胞胞浆抗体（ANCA）阳性小血管炎的临床病理表现及治疗。方法：对我院近年诊治的5例FTU引起的ANCA阳性小血管炎患者进行临床病理分析及治疗效果回顾。结果：5例病人中男1例，女4例；年龄11岁～39岁，平均26岁；服PTU时间3～36个月，4例有肾脏受累，3例肺部受累，其他如血液系统、皮肤、关节肌肉、眼鼻等均可受累。ANCA检测5例均识别MPO，ESR明显增快，CRP增高。肾活检1例新月体肾炎，1例局灶节段肾小球硬化症，1例局灶性坏死性肾小球肾炎。5例患者立即停服PTU，均应用免疫抑制荆，2例咯血合并感染者加用丙种球蛋白。5例患者随访7～43个月，临床症状缓解，ANCA检测阴性。结论：PTU可引起ANCA阳性小血管炎，及时停PTU，并予免疫抑制剂治疗，预后好。     

血管炎患者抗中性粒细胞胞浆抗体检测的意义

了解血管炎的发病机理、诊断和分类。方法采用间接免疫荧光法（ＩＦ）和酶联免疫吸附试验（ＥＬＩＳＡ）对５０例各类血管炎作血抗中性粒细胞胞浆抗体（ＡＮＣＡ）的定性及定量检测，同时应用ＰＥＧ－紫外分光定量法作循环免疫复合物（ＣＩＣ）水平测定。结果在结节性血管炎和变应性血管炎特别是前者的发病中，ＡＮＣＡ可能起较主要的作用，ＡＮＣＡ水平升高且定性呈阳性尤其是核周型ＡＮＣＡ阳性者，可能代表一组伴肾脏受累的血管炎患者。结论通过ＡＮＣＡ定性、定量及ＣＩＣ水平的检测，有助于血管炎的分类及诊断，并可用于判断器官受累的程度及评价治疗效果。     

狼疮性肾炎患者血清DNA－抗－DNA免疫复合物与蛋白尿和肾脏病理类型的关系

利用单克隆抗－ＤＮＡ抗体检测了３９名狼疮性肾炎（ＬＮ）患者不同的时期内不同类型的血清ＤＮＡ－抗－ＤＮＡ免疫复合物（抗ＤＮＡＩＣ），其中２４例做了肾活检。结果显示：活动期ＬＮ患者血清抗－ＤＮＡＩＣ的类型分布与患者的肾脏病理类型有一定的关系，有助于推测ＬＮ肾组织病理类型，ＩｇＧ型的抗单链ＤＮＡＩＣ（抗－ｓｓＤＮＡ－ＩｇＧ）与２４ｈ尿蛋白定量呈正相关。进一步说明抗－ＤＮＡＩＣ在ＬＮ的发病机理中有较重要的作用。     

ANCA相关性血管炎的治疗进展

ANCA(抗中性粒细胞胞浆抗体,anti-neutrophil cytoplasm antibodies)相关性血管炎常包括韦格纳肉芽肿(Wegener's granulomatosis,WG)、显微镜下多血管炎(microscopic polyangiitis,MPA)及局限于肾脏的血管炎,以血循环中存在抗中性粒细胞胞浆抗体为主要特征,可导致快速进展型肾小球肾炎(RPGN),其共同的肾脏病理表现为新月体的形成.如不及时治疗,则能在短期内进展为终末期肾衰竭(ESRD),近年来针对ANCA相关性血管炎的治疗有较大进展,现综述如下.     

Propylthiouracil-Induced Vasculitis Associated with ANCA: A Case Report

Propylthiouracil is a drug used in the treatment of hyperthyroidism for more than 60 years. Adverse side effects are seen in 1–5% of patients. Renal complications of the drug including glomerulonephritis and vasculitis are rarely seen. Cases of propylthiouracil-induced rapidly progressive glomerulonephritis and vasculitis are reported in association with antineutrophil cytoplasmic autoantibodies. Here we report a case of positive antineutrophil cytoplasmic autoantibodies rapidly progressive glomerulonephritis (RPGN) associated with propylthiouracil treatment.     

中性粒细胞细胞外网络在ANCA相关性小血管炎患者肾组织中的表达及意义

目的:研究中性粒细胞细胞外网络(NETs)在ANCA相关性小血管炎(AASV)患者肾组织中的表达并初步探讨其在AASV中的可能致病机制。方法:采用免疫组织化学技术,分别检测NETs(以瓜氨酸化的组蛋白H3为标志)在8例ANCA相关性小血管炎患者,8例微小病变型肾病综合征患者,3例健康对照的肾小球、肾间质及肾小管的表达,同时检测相应部位B淋巴细胞(CD19做标志)的浸润。结果:(1)AASV患者肾小球中NETs表达较微小病变组患者和健康对照组肾小球中NETs表达明显增多[(0.354±0.347)vs(0±0),P<0.05],在AASV肾脏病理损害严重部位,如中、重度系膜细胞增生的肾小球(0.485±0.721),有炎症细胞浸润的肾间质(2.575±2.99)、肾小管(1.417±2.888)中浸润的NETs明显增多。(2)B淋巴细胞(CD19)的表达:AASV患者肾小球球周B淋巴细胞表达较健康对照组[(3.123±4.411)vs(0±0)]、微小病变组[(3.123±4.411)vs(0±0)]表达明显增多,与微小病变组相比差异有统计学意义(P<0.05)。在AASV患者肾组织中主要分布于硬化的肾小球球周(4.024±7.457)、中重度系膜细胞增生的肾小球球周(2.673±2.948),在肾间质炎细胞浸润的区域常成簇聚集(9.625±8.961),而在肾小球内基本无表达。结论:NETs在AASV患者肾组织广泛表达,肾小球中浸润的NETs可能参与了AASV患者的肾损害。     

Humoral and Cellular Immune Responses after Influenza Vaccination in Kidney Transplant Recipients

It has been speculated that influenza vaccination of renal allograft recipients could be associated with de novo production and/or increased titers of anti-HLA antibodies (HLA-Ab). To directly address this issue, we recruited 66 stable renal transplant recipients and 19 healthy volunteers during the 2005–2006 vaccination campaign. At day 0 and day 30 following vaccination, HLA-Ab were screened and in parallel influenza-specific antibody and T-cell responses were assessed. Humoral postvaccinal responses to A/H1N1 and A/H3N2 strains, but not B strain, were less frequent in transplanted patients than in control subjects. Significant expansion of influenza-specific IFN-γ-producing T cells was observed at similar frequencies in patients and controls. There was no correlation between cellular and humoral postvaccinal responses. No impact of sex, age or immunosuppressive regimen could be evidenced. Vaccination was not associated with any significant change in preexisting or de novo anti-HLA sensitization. No episode of allograft rejection was recorded in any of the patients. Our results suggest that flu vaccination is safe in stable renal transplanted patients. Larger studies are needed for definitive statistical proof of the safety and effectiveness, with regard to the quality of the immune response, of yearly influenza vaccination in immunosuppressed patients.     

Clinical and Pathological Characterization of Patients with Microscopic Polyangiitis with Medium Artery Involvement

Objective.?To study the clinical and pathological characteristics of patients with microscopic polyangiitis (MPA) with medium artery involvement. Methods. Hospitalized patients with MPA in recent two years were retrospectively studied. Their clinical and pathological features were compared between patients with and without renal medium artery involvement. Results.?Thirty-nine patients had renal pathology confirmed MPA. Nine cases were with medium artery involvement. For the 30 patients without medium artery involvement, 24/30 had crescentic glomerulonephritis and 11/30 also had focal segmental glomerular fibrinoid necrosis; clinically, 21/30 patients were pANCA/MPO-ANCA positive, 26/30 had acute renal failure with an average duration of 14 weeks before MPA was diagnosed, eight cases achieved complete remission after intensive immunosuppressive therapy. Nine MPA patients had medium artery involvement, manifested as segmental fibrinoid necrosis of major branch of arcuate artery, glomerulus ischemia was predominant, but crescentic lesions were mild, none of them reached crescentic glomerulonephritis. Five of the nine were pANCA/MPO-ANCA positive, eight out of the nine patients had acute renal failure with an average duration of eight weeks before diagnosed, seven of the nine achieved complete remission after intensive immunosuppressive therapy. In comparison, MPA with medium artery involvement had a shorter duration (p < 0.05), less crescentic glomerulonephritis in patients with acute renal failure (p<0.01) and more patients achieved complete remission after treatment (p<0.05). Conclusion.?In present study, about 23% MPA patients had medium artery involvement and their impaired renal function is mainly due to extensive glomerular ischemia. These patients progressed to acute renal failure quicker and responded to therapy better.     

马蹄肾合并肾小球病的临床病理分析

也页目的：马蹄肾是先天性肾脏融合畸形,马蹄肾合并肾小球病时,由于担心肾穿刺术后并发症,很少进行肾活检,从而得不到明确的病理诊断,难以得到应有的正确治疗。本文通过总结分析马蹄肾合并肾小球病的临床病理资料,旨在提高其诊断与治疗水平。方法：收集2008年1月~2013年6月在解放军总医院就诊,通过影像学确诊马蹄肾且肾活检病理诊断为肾小球病的病例。回顾分析这些病例的临床资料、实验室检查、影像学检查、肾活检适应证的把握、肾穿刺注意事项、肾活检病理检查结果以及治疗随访的资料。结果：共5例患者影像学检查确诊马蹄肾且肾活检病理诊断为肾小球病。（1）临床上主要表现为夜尿增多、下肢水肿、高血压及尿检异常。（2）尿蛋白定量均〉1 g/24 h,血肌酐正常或偏高。（3）影像检查明确诊断为马蹄肾,均为下极融合。（4）均有肾活检的适应证,没有肾脏大血管畸形等禁忌证,患者血压均控制在140/90 mmHg以下,凝血功能正常;在患者签署知情同意书后,由经验丰富的医生在超声引导下用肾穿刺活检枪在“肾上极”进行肾活检;肾穿刺术后严密观察,患者没有出现术后并发症。（5）肾活检病理诊断分别为局灶节段性肾小球硬化、膜性肾病、原发性IgA肾病、过敏性紫癜肾炎（继发性IgA肾病）、狼疮肾炎。（6）根据肾活检病理结果给予相应的治疗,每月随访1次,随访6个月后结果显示患者尿蛋白定量明显下降、血压及血肌酐趋于稳定。结论：肾小球病是马蹄肾的重要合并症,充分评估其肾活检的意义及风险,明确其病理类型,有助于指导治疗、延缓疾病的进展。     

Utility of Intravenous Immune Globulin in Kidney Transplantation: Efficacy, Safety, and Cost Implications

Intravenous immunoglobulin preparations (IVIG) are known to be effective in the treatment of various autoimmune and inflammatory disorders into their immunomodulatory, immunoregulatory, and anti-inflammatory properties. Recently, IVIG has been utilized in the management of highly sensitized patients awaiting renal transplantation. The mechanisms of suppression of panel reactive antibodies (PRA) in patients awaiting transplantation are currently under investigation and appear to be related to anti-idiotypic antibodies present in IVIG preparations. In this review, the various immunomodulatory mechanisms attributable to IVIG and their efficacy in reducing PRAs will be described. In addition, the use of IVIG in solid organ transplant recipients will be reviewed. The adverse events, safety considerations, and economic impact of IVIG protocols for patients awaiting solid organ transplantation will be discussed.     

男性狼疮性肾炎的临床与病理特征

目的：提高对男性狼疮肾炎（LN）临床与病理特征的认识.方法：对28例男性LN与同期28例随机选择的女性LN从临床表现、病理学、免疫学特征、治疗及预后进行回顾性分析.结果：男性LN较易误诊（P＜0.01）和延迟诊断（P＜0.05）,肾衰竭发生率和需要冲击治疗者多于女性LN（P＜0.05）,蝶形红斑、关节痛、口腔溃疡较女性少见（P＜0.05）,ANA阳性率明显低于女性（P＜0.05）.病理类型男性Ⅳ型较女性多见,而Ⅱ型较女性少见.在随访1年且资料完整的患者中,女性LN完全缓解或部分缓解的比例明显多于男性LN（P＜0.05）.结论：男性LN与女性LN的表现有区别,且病情较重,预后差.     

70例男性系统性红斑狼疮患者临床及肾脏病理分析

目的：探讨男性系统性红斑狼疮（SLE）患者的临床及肾脏病理特点。方法：回顾性分析了我院2003年4月～2009年4月收治的70例男性SLE患者的临床表现及肾脏病理,并对部分患者进行了随访。结果：70例男性SLE患者年龄16岁～72岁,平均（34.8±14.1）岁,临床表现肾病综合征25例、慢性肾炎19例、急性肾衰竭8例、隐匿性肾炎14例,以及尿常规检查和肾功能正常者4例。42例患者进行了肾活检,肾脏病理结果为Ⅱ型3例、Ⅲ型5例、Ⅳ型20例、Ⅴ型5例,Ⅲ＋Ⅴ型4例,Ⅳ＋Ⅴ型5例。在肾病综合征患者中21例进行了肾活检的,病理类型分别为Ⅲ型1例、Ⅳ型9例、Ⅴ型4例、Ⅲ＋Ⅴ型2例、Ⅳ＋Ⅴ型5例;在8例急性肾衰竭患者中,7例进行了肾活检,病理均为Ⅳ型。结论：男性SLE在各个年龄段均可患病,临床和肾脏病理表现多样,多数患者病情较重,但也有部分患者肾脏损害较轻。