Characteristics of patients with coexisting IgA nephropathy and membranous nephropathy

Background: Coexistence of IgA nephropathy (IgAN) and membranous nephropathy (MN) in the same patient is rare. Few studies have reported the clinical and pathological features of patients with combined IgAN and MN (IgAN–MN).

Methods: The clinico-pathological features, levels of galactose-deficient IgA1 (Gd-IgA1) and autoantibodies against M-type transmembrane phospholipase A₂ receptor (anti-PLA₂R) in sera were compared among IgAN–MN, IgAN, and MN patients.

Results: Twenty-six patients with biopsy-proven IgAN–MN were enrolled. The mean age at biopsy was 43.6?±?15.9?years, and 65.4% were male. Proteinuria and estimated glomerular filtration rate (eGFR) levels in patients with IgAN–MN were similar to that of MN patients. Compared with the IgAN patients, IgAN–MN patients showed a higher median proteinuria level (4.3 vs. 1.2?g/day, p?2, p?IgAN–MN patients presented with milder pathological lesions than IgAN patients according to the Oxford Classification. IgAN–MN patients had comparable serum levels of Gd-IgA1 with those of IgAN patients (353.4?±?95.5 vs. 347.0?±?109.6?U/mL, p?=?.801). Percentage of IgAN–MN patients with detectable serum levels of anti-PLA₂R was lower than that of MN patients (38.5% vs. 68.6%, p?=?.011).

Conclusions: IgAN–MN patients display similar clinical features to MN patients and milder pathological lesions than IgAN patients. IgAN–MN patients have similar levels of Gd-IgA1 to those of IgAN patients, and a lower proportion of anti-PLA₂R than MN patients.     

End-Stage Renal Disease and Mortality Outcomes Across Different Glomerulonephropathies in a Large Diverse US Population

Objective

To compare renal function decline, incident end-stage renal disease (ESRD), and mortality among patients with 5 common glomerular diseases in a large diverse population.

New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch-Schönlein purpura)

Immunoglobulin A vasculitis (IgAV), also referred to as Henoch-Schönlein purpura, is the most common form of childhood vasculitis. The pathogenesis of IgAV is still largely unknown. The disease is characterized by IgA1-immune deposits, complement factors and neutrophil infiltration, which is accompanied with vascular inflammation. Incidence of IgAV is twice as high during fall and winter, suggesting an environmental trigger associated to climate. Symptoms can resolve without intervention, but some patients develop glomerulonephritis with features similar to IgA nephropathy that include hematuria, proteinuria and IgA deposition in the glomerulus. Ultimately, this can lead to end-stage renal disease. In IgA nephropathy immune complexes containing galactose-deficient (Gd-)IgA1 are found and thought to play a role in pathogenesis. Although Gd-IgA1 complexes are also present in patients with IgAV with nephritis, their role in IgAV is disputed. Alternatively, it has been proposed that in IgAV IgA1 antibodies are generated against endothelial cells. We anticipate that such IgA complexes can activate neutrophils via the IgA Fc receptor FcαRI (CD89), thereby inducing neutrophil migration and activation, which ultimately causes tissue damage in IgAV. In this Review, we discuss the putative role of IgA, IgA receptors, neutrophils and other factors such as infections, genetics and the complement system in the pathogenesis of IgA vasculitis.     

Up-regulation of CX3CR1 on tonsillar CD8-positive cells in patients with IgA nephropathy

Although tonsillectomy are used as therapeutic options to prevent chronic renal failure in IgA nephropathy (IgAN) patients, the relationship between IgAN and tonsils is not fully proved by basic research. Recently, circulating CX3CR1-positive cells were reportedly involved in promoting hematuria in patients with IgAN. In this study, we focused on the expression of CX3CR1 in tonsillar mononuclear cells in IgAN patients. Immunohistological analysis revealed greater distribution of CX3CR1-positive cells in the inter-follicular area of tonsils in IgAN patients than in non-IgAN patients. CX3CR1-positive cells were also found in the affected renal glomerulus of IgAN patients. Flow cytometric analysis revealed the expression of CX3CR1 on tonsillar CD8-positive cells to be significantly higher in IgAN patients. CpG-oligodeoxynucleotides enhanced the expression in IgAN patients. The chemotactic response of tonsillar mononuclear cells to fractalkine was significantly higher in IgAN patients. Expression of CX3CR1 on peripheral blood CD8-positive cells in IgAN patients was significantly higher, and decreased after tonsillectomy, along with the disappearance of hematuria. These results suggest that hyper-immune response to microbial DNA enhanced the expression of CX3CR1 on tonsillar CD8-positive cells in IgAN patients, followed by the migration of the cells to renal lesions via blood circulation, resulting in the development of hematuria.     

488例IgA肾病中医证型与病理分级相关性分析

目的:总结分析我院肾病中心IgA肾病患者中医证型和病理分级的相关性,为中西医结合规范化诊治IgA肾病提供依据。方法:收集近9年我院行肾穿刺活检的488例IgA肾病患者的中医四诊资料和病理检查资料,对中医证型和Lee氏病理分级进行分析。结果:488例IgA肾病患者主证中,阴虚证与气阴两虚证病理变化相对较轻,以Lee氏Ⅱ、Ⅲ级为主(86.2%,90.2%);脾肾两虚证病理变化相对较重,以Lee氏Ⅲ、Ⅳ级为主(85.1%)。兼证中兼湿热者204例(41.8%),其次为兼浊毒者44例(9.0%)。488例患者中有233例主要表现为脾气虚证,并且以Lee氏Ⅱ、Ⅲ级为主(91.0%)。结论:中医辨证分型与肾脏病理分级有一定的相关性;而主要表现为脾气虚证患者占有很大比例。     

IgA肾病患者红细胞免疫功能测定的意义

摘要目的 研讨IgA肾病红细胞免疫状态及其意义。方法 46例经肾穿刺活检明确诊断的IgA肾病,运用流式细胞仪检测红细胞膜表面CD35活性水平。结果 随着病变加重,红细胞免疫功能下降也明显,IgA肾病CD35与24小时尿蛋白定量无显著相关性,但与血清肌酐、系膜增殖程度、肾小球硬化呈正相关性(r=0.552,P<0.05,r=0.561,P<0.05,r=0.637,P<0.01,)。结论 红细胞免疫功能紊乱可能是影响IgA肾病进展的因素之一。     

Ⅳ型胶原的表达及血管病变在IgA肾病中的意义

目的探讨Ⅳ型胶原的表达及血管病变在IgA肾病中的意义。方法采用特异性抗体及免疫组织化学染色方法,对经肾活检诊断的30例不同病理改变的IgA肾病患者肾组织中的Ⅳ型胶原的表达及血管病变与临床指标(病程、血压、肾功能改变)进行分析。结果在IgA肾病患者肾组织中,肾小球内Ⅳ型胶原随病变加重表达增多,在重度病变组表达最多(P0.01);随病理改变程度加重,血管病变也加重。多因素分析显示高血压、年龄、病理分级Ⅲ级以上(Lee分级)对血管病变的发生影响有显著意义。结论在IgA肾病中Ⅳ型胶原的表达及血管病变与血压、尿蛋白定量、血肌酐水平等临床指标能够反映病变的程度,可以作为判断预后及干预治疗的一个重要病理指标。     

基于真实世界的加味黄芪赤风汤治疗IgA肾病的临床疗效评价

目的:观察加味黄芪赤风汤治疗Ig A肾病蛋白尿的疗效并探讨BMI水平对其疗效的影响。方法:采用真实世界研究的方法,对2015年1月至2016年12月中国中医科学院西苑医院就诊的Ig A肾病并符合气虚血瘀证的患者295例,以加味黄芪赤风汤治疗24周;以BMI水平分层,探讨其对不同BMI水平患者疗效是否有差异。结果:治疗后24 h蛋白尿水平及中医症状积分下降,与治疗前比较,差异有统计学意义(P 0. 01); 24 h蛋白尿水平方面:总有效率达77%; BMI达标患者的有效率达82. 4%,超重患者有效率为72. 4%,经χ2检验,差异有统计学意义(P 0. 05);中医症状方面:有效率达72%;以BMI分层,2组有效率经统计学分析,差异无统计学意义(P 0. 05)。结论:在真实的临床条件下,加味黄芪赤风汤对气虚血瘀型Ig A肾病蛋白尿患者的治疗效果显著; BMI水平可能影响Ig A肾病患者的恢复治疗。     

HIV感染合并IgA肾病4例临床和病理分析

目的 研究HIV感染合并IgA肾病患者的临床和病理特点及治疗转归.方法 选择2002-2012年在北京协和医院住院的HIV感染合并肾脏损害,并经肾脏活检证实为IgA肾病的4例患者为研究对象,回顾分析这4例IgA肾病患者的临床、病理资料及治疗随诊情况.结果 4例患者均有不同程度蛋白尿和镜下血尿,血肌酐水平在正常范围,肾脏活检病理提示为不同程度的IgA肾病.4例患者均接受了高效联合抗反转录病毒治疗(HAART).3例患者的初始治疗使用了ACEI/ARB,其中2例效果不佳联合使用糖皮质激素治疗.1例肾脏病变好转后再次出现大量蛋白尿,考虑与抗病毒药物替诺夫韦有关,调整抗病毒治疗后再次缓解.治疗过程中所有患者的HIV病毒复制并未增加.结论 HIV感染合并肾脏病变除了经典塌陷型FSGS之外还可以合并IgA肾病,肾脏活检有利于明确诊断并指导治疗.