伴毛细血管袢纤维素样坏死的原发性IgA肾病患者的临床及病理特征及其随访观察

目的:探讨伴毛细血管袢纤维素样坏死的原发性IgA肾病患者的临床病理特征及预后情况。方法:回顾分析2009年~2014年在本院确诊的74例原发性Ig A肾病合并毛细血管袢纤维素样坏死(坏死组)的临床与病理资料,采用配对方法随机选取82例同期确诊为原发性IgA肾病不伴有毛细血管袢纤维素样坏死(非坏死组)为对照,中位数随访时间26个月。比较两组患者的临床病理特征及预后情况,主要终点事件定义为血肌酐上升50%或ESRD,次要终点事件为蛋白尿缓解率。采用Kaplan-Meier方法进行生存分析。结果:基线时坏死组尿蛋白水平、尿白蛋白与肌酐比值、肉眼血尿发生率、新月体发生率、新月体百分比均高于非坏死组(P0.05),但球性硬化比率低于非坏死组(P=0.04)。主要终点及次要终点事件的例数均以坏死组为多,经Kaplan-Meier方法进行生存分析,两组主要及次要终点事件未达显著差异。结论:伴毛细血管袢纤维素样坏死的原发性IgA肾病患者新月体及肉眼血尿发生比率高,尿蛋白水平高,球性硬化程度相对较轻。毛细血管袢纤维素样坏死对IgA肾病短期预后无显著影响,需进一步随访明确其长期预后。     

MCP-1在IgA肾病肾组织中的表达与临床病理关系

目的 研究MCP-1在人类IgA肾病肾组织中的表达,进一步了解MCP-1与IgA肾病临床病理关系,并探讨其在IgA肾病进展中的可能作用机制.方法 根据IgA肾病病理半定量评分系统中8个病理指标评分标准,给予IgA肾病组按照病变程度赋予分值,采用SP免疫组织化学方法检测正常对照组和Iga肾病组肾组织中MCP-1的表达,探讨MCP-1与IgA肾病临床病理关系.结果 (1)在正常肾组织中,MCP-1表达较弱,正常对照组与IgA肾病组肾组织中MCP-1表达量均有统计学差异(P＜0.05);(2)IgA肾病肾组织中MCP-1表达量与IgA肾病组织学半定量评分法8个病理指标中破坏肾小球毛细血管袢活动性指数(dGAI)、系膜细胞增生指数(MsHI)、系膜基质增多指数(MsMI)、肾小球慢性病变指数(GCI)、肾间质炎症细胞浸润指数(infI)、肾小管萎缩和肾间质纤维化指数(TCI)等6个指标正相关(P＜0.05),而与内皮细胞增生指数(endol)、小动脉慢性病变指数(VCI)无明显相关性(P＞0.05);IgA肾病肾组织中MCP-1表达量与患者的蛋白尿水平正相关、肌酐清除率水平负相关(P＜0.05),而与患者血尿水平无明显相关性(P＞0.05).结论 在IgA肾病组肾组织中,MCP-1表达量与反映肾小球硬化、肾小管间质纤维化和细胞外基质积聚的病理指标呈正相关,提示其在IgA肾病病情进展中起重要作用.在IgA肾病组肾组织中,MCP-1表达量与蛋白尿呈正相关、肌酐清除率呈负相关,提示其与IgA肾病肾功下降、预后不良有关.     

IgA肾病患者合并高尿酸血症临床及病理特征分析

目的探讨IgA肾病高尿酸血症的临床病理特征及相关危险因素。 方法选取2010年1月至2015年7月于山西医科大学第二医院行经皮肾穿刺活检确诊为IgA肾病的188例患者,根据血尿酸水平将患者分为正常血尿酸组与高尿酸血症组,收集患者一般资料、尿蛋白定量、肾功能等临床生化指标以及病理指标,并对肾脏病理组织进行牛津病理分型。分析两组患者临床表现、肾脏病理特点,应用多元回归统计学方法分析高尿酸血症发生的影响因素。其他数据采用SPSS13.0软件进行统计分析。 结果本研究中心188例IgA肾病患者中合并高尿酸血症的患者有42例,高尿酸血症发生率为22.3%;高尿酸血症组中男性患者36例,女性患者6例;与正常血尿酸组(男性患者61例、女性患者85例)相比,高尿酸血症组男性患者明显增多(χ²＝25.2,P<0.001)。本组研究IgA肾病患者肾脏组织牛津病理分型以M1E1S0T0多见;与正常血尿酸组比较,高尿酸血症组患者肾小管－间质损伤重,差异有统计学意义(χ²＝5.056,P＝0.025)。肾组织免疫复合物IgA沉积于毛细血管袢者高尿酸血症发生率明显升高(χ²＝44.69,P<0.001)。IgA肾病患者合并高尿酸血症的相关因素为性别、体质量指数、甘油三酯、IgA沉积于毛细血管袢。 结论IgA肾病高尿酸血症的危险因素为男性、肥胖、高甘油三酯血症,并可能与IgA在毛细血管区沉积相关。     

伴毛细血管襻纤维素样坏死IgA肾病的临床病理特点及预后分析

目的:回顾性分析伴毛细血管襻纤维素样坏死局灶增生性IgA肾病(IgAN)的临床病理特点及预后,探讨纤维素样坏死在IgAN的意义。方法:对55例伴纤维素样坏死局灶增生性IgAN患者的临床、实验室、病理表现及预后进行回顾性分析;并与同期60例不伴有纤维素样坏死的局灶增生性IgAN进行比较。结果:两组患者临床均可表现为隐匿性肾小球肾炎,肾炎综合征及肾病综合征。坏死组近一半的患者有前驱感染史及血IgA升高,多数患者24 h尿蛋白定量大于1 g,常伴发作性肉眼血尿。病理方面,坏死组伴发局灶新月体形成更常见。随访结束时,两组患者的肾脏生存率在随访期内差异无统计学意义。结论:伴纤维素样坏死的局灶增生性IgAN与对照组比较,尿蛋白大于1 g,前驱感染,发作性肉眼血尿及血IgA升高更常见。可出现肾功能异常。经积极治疗预后与非坏死组比较差异无统计学意义。     

2型糖尿病合并非糖尿病肾病的临床病理特征和预测因子的分析

目的:探讨2型糖尿病(type 2 diabetes mellitus, T2DM)合并非糖尿病肾病患者的临床指标和肾活检病理特征,寻找疾病的预测因子。方法:回顾性分析2016年07月—2021年12月在我院行肾活检的76例T2DM患者病例。根据活检结果分为糖尿病肾病(diabetic nephropathy, DN)组30例,非糖尿病肾病伴/不伴糖尿病肾病(non-diabetic renal disease with/without diabetic nephropathy, NDRD±DN)组46例,对其临床指标及病理结果进行分析。结果:两组患者的年龄、性别、糖尿病病程(diabetes mellitus, DM)、糖尿病视网膜病变(diabetic retinopathy, DR)、糖化血红蛋白、血红蛋白、血肌酐、尿素氮和胱抑素C比较差异有统计学意义(P<0.05)。肾活检常见原因有活动性尿沉渣,其次为DM<5年且无大量蛋白尿和新出现的肾病综合征。NDRD±DN组最常见的病理类型是膜性肾病,IgA肾病次之。二元Logistic回归分析发现性别、年龄、DR、糖化血红蛋...     

IgA肾病患者尿足细胞排泄与临床病理相关性分析

目的观察原发性IgA肾病患者尿足细胞排泄、肾小球足细胞病变,分析其与临床病理之间的关系。方法50例经肾活检明确诊断的IgA肾病患者和10名健康志愿者,利用podocalyxin（PCX）作为标记蛋白,标记尿液和肾组织足细胞,收集患者肾活检时临床资料,各项病理指标和肾组织足细胞PCX荧光表达采用不同的半定量积分法进行评分,电镜检测肾小球外周袢的足突宽度。结果①IgA肾病患者中尿足细胞染色阳性为32例（64％）,较健康对照者有统计学差异。②IgA肾病伴尿足细胞阳性患者尿蛋白水平、血肌酐（SCr）、平均动脉压（MAP）较尿足细胞阴性患者增高,血浆白蛋白（Alb）、肾小球滤过率（GFR）降低（P〈0．05）。③光镜示IgA肾病伴尿足细胞阳性患者肾小球硬化程度、新月体发生率较尿足细胞阴性患者明显增高（P〈0．05）,但小管间质病变与肾组织足细胞PCX表达阳性指数,2组比较无统计学差异。④电镜结果提示IgA肾病患者尿足细胞阳性足突宽度明显增宽（P〈0．05）。结论足细胞尿是反映肾脏疾病轻重的一个指标,足细胞尿与肾脏病理类型有一定关系,IgA肾病患者尿足细胞排泄指标是否能够独立预测患者的预后还有待证实。     

重复肾活检对IgA肾病治疗效果的观察

目的:通过重复肾活检观察IgA肾病患者的病理改变.方法:将12例IgA肾病患者行重复肾活检,观察2次肾活检时临床和实验室指标、肾组织病理积分以及系膜区IgA荧光强度的变化.结果:治疗后尿24 h蛋白定量明显减少(P＜0.001),血IgA水平降低(P＜0.05),但血肌酐、内生肌酐清除率、血压水平无统计学差异(P＞0.05);2次肾活检比较,系膜增生程度明显减轻(P＜0.001),节段硬化和球囊黏连明显减少(P＜0.001),肾间质细胞浸润明显减少(P＜0.01),新月体全部消失,间质纤维化程度减轻(P＜0.001);球性硬化无统计学差异(P＞0.05);系膜区IgA荧光强度明显减弱.结论:IgA肾病进行早期治疗,不仅临床治愈,组织学也可逆转.     

内蒙古地区汉族IgA肾病患者PAI-1基因多态性与临床病理特征的关系

目的:研究影响内蒙古地区汉族IgA肾病患者PAI-1基因多态性与临床病理特征的关系。方法:采集100例肾活检证实的IgA肾病患者血样,提取基因组DNA。用等位基因特异聚合酶链反应(ASPCR)法进行PAI-14G/5G基因型分析。分别分析各基因型与IgA肾病患者的临床特征、病理类型的关系。结果:IgA肾病组的4G/4G基因型发生频率(41%)显著高于对照组(23%,P值<0.01)。血尿的分级水平在4G/4G基因型患者中显著高于4G5G或5G5G基因型患者(χ2=6.71,P<0.05)。PAI-1基因型频率和等位基因频率与IgA肾病的病理Hass分级无关(P>0.05),伴肾小球硬化组的4G/4G基因型频率显著高于不伴肾小球硬化组(P<0.05)。结论:PAI-14G/4G基因型是内蒙古地区汉族IgA肾病患者发病的易感基因型,除血尿的分级水平外PAI-1基因的三种基因型的临床特征差异无统计学意义。PAI-1基因4G/5G多态性与IgA肾病肾小球硬化显著相关,而与其病理Hass分级无关。     

正常血压IgA肾病患者动脉病变的意义

目的:传统观点认为动脉硬化与高血压有关。然而,部分IgA肾病患者,尽管血压正常,但仍有动脉硬化的改变。本研究的目的就是比较血压正常、伴有和不伴有动脉病变的IgA肾病患者的临床病理特点,探讨正常血压IgA肾病患者肾内动脉病变的影响因素及意义。方法:所有患者均经肾活检诊断为原发性IgA肾病,无高血压病史,肾活检前血压<140/90mmHg。动脉病变的定义为活检肾组织光镜下见动脉壁增厚和(或)动脉玻璃样变。符合标准的105例患者,根据动脉病变的有无分为两组,有动脉病变组52例、无动脉病变组53例,分别比较两组的临床病理特点。肾脏动脉病变的半定量分级标准:0:无损害;1:<25%;2:≥25%,<50%;3:≥50%。统计学方法:分别比较两组的临床病理特点,将差异有统计学意义的单因素指标作为多因素分析的入选指标,采用逐步回归方法分析动脉病变的影响因素,以P<0.05作为差异有统计学意义。结果:与无动脉病变组比较,动脉病变组肾活检时的年龄、血肌酐、血尿酸、尿蛋白定量、尿NAG酶、肾小球硬化、肾小管萎缩以及肾间质纤维化的程度显著增高,尿渗透压显著下降。多因素分析的结果表明,肾活检时的血肌酐、尿渗透压、肾小管萎缩及肾间质纤维化是正常血压IgA肾病动脉病变的独立影响因素。动脉病变的程度与血肌酐、肾小管萎缩及肾间质纤维化呈正相关;与尿渗透压呈负相关。结论:血压正常IgA肾病患者的肾内动脉病变,主要与年龄、血肌酐、血尿酸增高等因素有关,常伴有肾小管间质损害。     

伴弥漫性毛细血管内增生的儿童原发性IgA肾病临床病理分析

IgA肾病(IgAN)是儿童常见的肾小球疾病,病理改变以系膜增生为主,弥漫性毛细血管内增生(DEP)较少报道.我们回顾性分析83例IgAN资料,比较伴或不伴DEP的临床与病理差异.一、对象与方法 1.对象:选择2000年1月至2011年6月在我院经临床、肾活检确诊为原发性IgAN且病历资料完整的83例患儿为对象,根据是否伴DEP分为非DEP组74例和DEP组9例.     

Idiopathic IgA nephropathy with segmental necrotizing lesions of the capillary wall

BACKGROUND: Segmental glomerular necrosis has been described in the biopsy material in a minority of patients with idiopathic IgA nephropathy in the oldest studies on this disease, but this marker of active capillaritis has received little attention in the subsequent literature, and its significance and relevance for the clinical outcome is still unknown. METHODS: Thirty-five out of 340 patients (10.3%) biopsied in our division at the San Carlo Hospital since 1974 showed active segmental necrotizing lesions. The morphological features and the natural history of this group of patients were compared with those of a control group of 229 patients who had comparable serum creatinine and extent of glomerular sclerosis, but who lacked active segmental necrosis. RESULTS: Patients with the necrotic variant showed a significantly more marked extracapillary proliferation and interstitial accumulation of monocytes and T lymphocytes and, in the segmental areas of necrotizing and extracapillary lesions, infiltration of monocytes, deposition of fibrinogen, and expression of the adhesion molecule vascular cell adhesion molecule-1. No difference was found in the presenting clinical syndrome. The clinical course was frequently characterized by acute flare ups, and the progression to end-stage renal failure was more frequent, although actuarial renal survival was not significantly worse (P = 0.07). The aggressive treatment with steroids and cyclophosphamide, carried out in 20 of the 35 patients, has probably been beneficial, justifying the multicenter controlled trial that recently has been initiated. CONCLUSIONS: Vasculitic lesions of the glomerular capillaries, with histologic and immunohistological features similar to those of Henoch-Sch?nlein purpura and antineutrophil cytoplasmic antibody-positive renal vasculitis, were found in 10% of patients with idiopathic IgAN. Clinical features at presentation did not differ from those of the other patients with IgAN, and despite of the more frequent occurrences of recurrent acute flare ups, rapid progression to end-stage renal failure was a rare phenomenon, even in untreated patients.     

合并毛细血管襻纤维素样坏死的原发性IgA肾病患者的预后

目的 初步探讨合并毛细血管襻纤维素样坏死的IgA肾病(IgAN)的临床病理特点及纤维素样坏死对IgAN近期预后的影响。方法 回顾分析1997年至2004年10月在北京大学第一医院肾内科经肾活检诊断资料完整的780例原发性IgAN。在46例伴有纤维素样坏死的患者中，35例有完整的随访数据，平均随访时间26个月。随机选取同期的80例无毛细血管襻纤维素样坏死的IgAN患者，平均随访时间39个月，比较两组患者的临床病理及预后情况。终点事件定义为Scr上升50%或Ccr下降33%或ESRD。采用Kaplan-Meier方法进行生存分析及Cox风险比例模型筛选预后危险因素。结果 坏死组肾组织的淋巴单核细胞浸润更为明显(P = 0.004)；肾小球硬化比率较低(P = 0.002)。坏死组中仅有2例以急性肾功能衰竭(ARF)起病，3例达随访终点；80例非坏死组患者中有14例达终点。多因素分析提示，纤维素样坏死不是IgAN预后的独立危险因素，而严重慢性化病变是唯一提示预后不良的危险因素(RR = 23.13， P < 0.01)。结论 合并纤维素样坏死的IgAN在临床病理方面并无显著特点，纤维素样坏死不影响IgAN的短期预后。     

Impact of gender on the clinicopathological features of patients with primary IgA nephropathy

Objective To explore the impact of gender on the clinicopathological features of patients with primary IgA nephropathy (IgAN). Methods All patients with IgAN who were biopsy-proven in The First Affiliated Hospital, Sun Yat-sen University from January 2006 to December 2011 were divided into two groups by gender: male group and female group. The clinical manifestations and pathological features of two groups were retrospectively investigated and compared. Results A total of 1512 primary IgAN patients were enrolled in the study, and the ratio of male to female was 1∶1.16, with a median age of 32(26, 39) years old at biopsy. Compared to female patients, male patients with IgAN exhibited more severe clinical manifestations including worse renal function, greater urinary protein excretion, and more frequent occurrence of hypertension, hypertriglyceridemia and hyperuricemia. Besides, male patients had worse histological lesions, including more severe segmental sclerosis, tubular-atrophy/interstitial fibrosis and interstitial infiltration. For female patients, hematuria, including gross and microscopic hematuria, was more frequent. Conclusion Male patients with IgAN were with worse clinicopathological changes than those of female.     

Idiopathic IgA nephropathy with diffuse crescent formation

OBJECTIVE: To investigate the clinicopathological features and outcome of idiopathic IgA nephropathy with diffuse crescent formation in Chinese patients. METHODS: Twenty-five patients with diffuse crescentic IgA nephropathy (DCIgAN), 15 males and 10 females with median age of 28.5, and median disease duration of 5.1 months, were studied. Their clinical, laboratory and pathological features and outcome were investigated. Twenty-one were administered pulse immunosuppressive therapy, and 15 were followed up for more than 6 months. RESULTS: 1.14% had total IgA nephropathy, and 16.4% total diffuse crescentic glomerulonephritis. Clinically, most of patients (88%) showed rapidly progressive glomerulonephritis associated with a high level of serum creatinine (418 +/- 264 micromol/l). Gross hematuria was noted in 72%, hypertension in 64%, and nephrotic syndrome in 48%. Pathologically, except for diffuse crescent formation (a median 65% and range 50-95%), we observed segmental necrosis of glomerular capillaries in 60%, glomerular infiltrating cells in 48%, endothelial cells proliferation in 32%, and rupture of Bowmans' capsule in 24%. Severe tubular interstitial damage was also found, tubular atrophy in 64%, interstitial fibrosis in 60%, diffuse interstitial infiltrating cells in 74%, and interstitial vasculitis in 40%. Immunopathologically, four phenotypes were observed; however, IgA associated with IgM deposition was higher than that in patients with general IgA nephropathy (IgAN). In addition, the infiltrating CD4+, CD8+, CD68+ and PCNA+ cells in renal tissue were significantly high compared with that in controls. In a follow-up study, 66.7% of patients had life-sustaining renal function, 4 of them had normal range of serum creatinine (<124 micromol/l), and only 5 were dialysis-dependent. CONCLUSIONS: The patients with crescentic IgA nephropathy mostly show rapidly progressive nephritis associated with more severe pathological changes including glomerular, tubular interstitial and vascular lesions than in patients with general IgAN. The infiltrates in glomeruli may contribute to the crescentic formation, and the intensive immune suppressing treatment is useful to improve renal damage in patients with DCIgAN.     

A case of mixed membranous nephropathy and purpura nephritis

We report the case of a 71-year-old man with mixed glomerular lesions, membranous and necrotizing changes. The patient had abdominal pain and purpurat on the extremities and trunk, followed by melena, and after admission to hospital, proteinuria and occult blood were noted. Laboratory findings were negative for autoimmune disease and viral hepatitis. Renal biopsy showed segmental necrotizing changes and mesangial proliferation with spike formation. Immunofluorescence revealed a granular deposition of IgA predominantly in the mesangial area in contrast to the granular IgG deposition along the glomerular capillary loops. Moreover, electron-microscopically, mesangial as well as subepithelial electron-dense deposits were observed. These data suggest that the patient had 2 distinct types of glomerulonephritis simultaneously: idiopathic membranous nephropathy and purpura nephritis.     

Evolution of renal injury in a chronic model of IgA immune-complex-associated nephropathy

BACKGROUND.: IgA nephropathy (IgAN) is characterized by intense and diffuseIgA mesangial deposits, a variety of histopathological changesand unpredictable clinical course. To elucidate the cause ofthe discrepancy between the unvariable IgA deposition and thehistological picture, we examined the short- and long-term influenceof glomerular IgA immune complexes (IgA-IC) on the progressionof renal lesions in experimental IgAN. METHODS.: IgA-IC renal deposits were induced by sequential administrationof IgA anti-phosphorylcholine and pneumococcal C polysaccharide.Mice treated every other day by three injections (groups A)or nine injections (groups B) were sacrificed 24 h and 1, 4,or 8 weeks (groups 1–4) after cessation of treatment. RESULTS.: Group A1 showed segmental glomerular necrosis and thrombosis.Lesions then converted to segmental mesangial proliferation(A2), more pronounced in A3 and minimal in A4. Group B1 showedsevere proliferative glomerulonephritis and segmental necrosis.The pattern altered to mesangial expansion with glomerular/interstitialinfiltration in B2, milder features in B3 and residual mesangialproliferation in B4. Proteinuria increased progressively duringtreatment reaching its maximum in group B1, but it returnedto near normal levels in group B4. The development of proteinuriaparalleled glomerular/interstitial T cell infiltration. CONCLUSIONS.: These findings demonstrate that renal histopathological alterationsobserved in experimental IgA nephropathy are sustainable onlyby continuous deposition of nephritogenic IgA-IC.     

尿足细胞阳性IgA肾病患者不同蛋白尿水平临床病理分析

目的：探讨尿足细胞阳性的IgA肾病患者不同蛋白尿水平的临床病理特点.方法：选取原发性IgA肾病尿足细胞阳性患者41例,收集患者临床病理资料,根据尿蛋白水平分成3组：组1（≤0.5 g/24 h）（n=9）,组2（〉0.5 g/24 h,〈1.0 g/24 h）（n=14）,组3（≥1.0 g/24 h）（n=18）,比较3组临床病理特点.结果：（1）临床特点：组1患者收缩压、eGFR、UA均较组3差异有统计学意义（P〈0.05）,且这些指标在三组间随蛋白尿水平呈现递进表现.组2和组3仅在收缩压、UA方面差异有统计学意义（P〈0.05）;（2）病理特点：组1在节段性肾小球病变比例及间质炎细胞浸润方面较组3轻,差异有统计学意义（P〈0.05）;组1在节段性肾小球病变比例及节段损伤积分方面较组2轻,差异有统计学意义（P〈0.05）;组2和组3两组病理改变差异无统计学意义（P〉0.05）.结论：尿足细胞阳性的IgA肾病患者中,尿蛋白水平仍然是肾脏病轻重的一个指标,与临床病理关系密切.     

Comparison of clinicopathological features between children and adults with IgA nephropathy

Background

IgA nephropathy (IgAN) is prevalent among both children and adults. Illumination of the differences between them is important for clinical doctors.

Methods

We retrospectively compared clinicopathological features in 110 children and 908 adults with IgAN.

Results

The male to female ratio was 1.62:1 in children and 0.85:1 in adults. Most patients lacked triggers, but IgAN was preceded by upper respiratory infection (URI) in 45.5% of children and 20.2% of adults. Gross hematuria was the most common initial symptom in children (53.6%), especially in those associated with URI (82.0%), while other symptoms and abnormal laboratory parameters were more common in adults. Estimated glomerular filtration rate (eGFR) was higher in children than in adults. Co-deposition of IgA and C3 were found in 50.9% of children, while IgA deposit was often accompanied by two or more immune complexes in adults. The frequency of subclass I was significantly higher in children than in adults. Mild histological lesions were more common in pediatric IgAN patients associated with URI than other patients.

Conclusions

Pediatric patients showed relatively mild clinical manifestations and histological lesions compared with adult patients. URI was the most important trigger for IgAN, particularly in children. IgAN associated with URI was relatively mild.     

贫血加重IgA肾病患者的临床和病理改变

目的 分析IgA肾病合并贫血患者的临床病理特征.方法 收集经肾活检确诊的IgA肾病患者临床资料409例,按照贫血与否分为非贫血组和贫血组,回顾性分析两组患者的临床和病理资料.结果 与非贫血组比较,贫血组患者的肾小球损伤和肾小管间质萎缩程度较重、24 h尿蛋白增多和eGFR降低.Spearman相关分析结果显示,血红蛋白、eGFR与肾脏病理损伤呈负相关(P＜0.05),血尿酸、24h尿蛋白与肾脏病理损伤呈正相关(P＜0.05).多因素Logistic回归分析发现贫血是肾小管间质萎缩的独立危险因素.结论 IgA肾病合并贫血患者的临床和病理损伤重于IgA肾病非贫血的患者,贫血参与IgA肾病的进展.     

Elevation of serum von Willebrand factor and anti-endothelial cell antibodies in patients with immunoglobulin A nephropathy are associated with intrarenal arterial lesions

Aim: Recent studies suggest that intrarenal arterial lesions are frequently observed in patients with immunoglobulin A nephropathy (IgAN). However, the mechanisms of the injury have not been elucidated. The level of serum von Willebrand factor (vWF) and the prevalence of anti-endothelial cell antibodies (AECA) were investigated in patients with IgAN with different intrarenal arterial lesions. Methods: Sera from 28 patients with mild intrarenal arterial lesions (group 1) and 36 patients with severe intrarenal arterial lesions (group 2) were collected. Sera from 20 patients with idiopathic membranous nephropathy (group 3) and 50 healthy volunteers were also obtained as disease and normal controls, respectively. Serum vWF and AECA of both IgG and IgA isotype were detected. Results: In comparison with normal controls, serum vWF was significantly higher in group 2 and group 3. Serum vWF was also significantly higher in group 2 than in group 1. Both IgG-AECA and IgA-AECA could be detected in three groups of patients. The prevalence of anti-87 kD IgG-AECA was greatest in patients in group 2. IgAN patients, especially those in group 2 with IgG-AECA or anti-87 kD IgG-AECA, had significantly higher serum creatinine and lower creatinine clearance than those without. No significant difference could be found for IgA-AECA. The level of serum vWF was higher in IgAN patients with IgG-AECA than that in patients without. Conclusion: Intrarenal arterial lesions are associated with endothelial cell damage in IgAN, and vWF is a useful serological biomarker of severe intrarenal arterial lesions. AECA, especially IgG-AECA, may play an important role in the pathogenesis of intrarenal arterial damage in IgAN.