IgA肾病的中医治疗现状

正IgA肾病是最早于1968年由法国学者Berger和Hinglais提出的免疫病理诊断,是指肾小球系膜区IgA沉积为主的原发性肾小球疾病~([1~2])。其主要表现为发作性或无症状性血尿,可伴或不伴有蛋白尿、高血压和肾功能受损。尽管经过各种治疗,每10年仍有20%的患者进入终末期肾病(ESRD)~([3])。其主要危险因素包括大量的蛋白尿、肾功能减退、肾小球硬化、肾     

邓跃毅教授治疗IgA肾病临床经验

<正>IgA肾病(IgA Nephropathy)是一种常见的原发性肾小球疾病,其特征是肾活检免疫病理显示在肾小球系膜区以IgA为主的免疫复合物沉积,以肾小球系膜增生为基本组织学改变。其临床表现多种多样,主要表现为血尿,可伴有不同程度的蛋白尿、高血压和肾功能受损,是导致终末期肾脏病的常见的原发性肾小球疾病之一。IgA肾病在全世界分布广泛,在亚洲和太平洋地区是最常见的原发性肾小球疾病,占肾活检     

预警IgA肾病进展的组织学及尿液标志物研究进展

IgA肾病是世界范围内最常见的原发性肾小球疾病,同时也是引起终末期肾功能衰竭的主要原因之一.IgA肾病临床以持续蛋白尿和(或)血尿为常见表现,病情变化及患者预后差异很大.早期确诊IgA肾病并筛选高危IgA肾病人群对于改善患者预后、制定合理的个体化治疗方案有蘑要意义.目前临床上主要通过检测尿蛋白多少、是否有高血压、肾功能...     

新疆维吾尔族原发性IgA肾病临床及病理特点分析

目的探讨新疆维吾尔族和汉族原发性IgA肾病临床及病理特点。方法对1999年6月至2005年12月在本院肾内科住院并经肾活检确诊的原发性IgA肾病患者的临床表现及病理特征进行回顾性分析。结果维吾尔族和汉族原发性IgA肾病患者分别为46、71例，分别占同期同民族原发性肾小球疾病的5．59％和6．59％；汉族与维吾尔族在年龄、性别、常见诱因及病理类型、免疫复合物沉积方式及部位等方面有相似之处。镜下血尿和轻度蛋白尿发生比例均高，起病时肾功能正常或伴轻度损害。不同之处：维吾尔族患者起病年龄相对较轻；维吾尔族以肾病综合征为常见，而汉族发作性肉眼血尿比例高。维吾尔族患者肾组织活动期病变及慢性化病变程度均轻于汉族。结论IgA肾病同样是维吾尔族青壮年男性中常见的原发性肾小球肾炎。     

IgA肾病中医辨证分型治疗新探

IgA肾病（IgAN）系指肾小球系膜区以IgA为主的免疫球蛋白沉积为免疫病理特征的一组肾小球疾病,是我国最常见的原发性肾小球疾病,约占肾活检病例的1／3,在确诊后5～25年内有20％-40％的患者可发展为慢性肾衰竭。IgA肾病临床表现多数以血尿为主,可伴有少量蛋白尿、大量蛋白尿或肾病综合征,少数病人可见急进性肾炎综合征。     

IgAN分子标记物的研究新进展表达及临床意义

<正>慢性肾脏病(chronic kidney disease,CKD)已成为全球性公共健康问题,包括一系列原发性和继发性肾小球肾炎,IgA肾病(immunoglobulinA nephropathy,IgA N)是目前我国最常见的原发性肾小球疾病,以IgA沉积在肾小球系膜区为特征的临床综合症。其临床表现差异较大,呈进行性发展,以血尿和(或)蛋白尿为主,同时伴不同程度的高血压和肾功能减退,任何年龄段均可发病,尤以青年多见。在我国,有25%~50%的IgAN患者     

IgA肾病分子机制研究进展

正IgA肾病是青壮年肾功能不全的重要病因,占我国原发性肾小球肾炎的20%～45%[1],该病是1968年由Berger和Hinglais提出的,临床表现主要为血尿,可伴有不同程度的蛋白尿、高血压和肾功能受损。免疫荧光病理表现为肾小球系膜区颗粒状IgA沉积,或伴有补体C3的沉积。有研究表明,IgA肾病患者接受肾移植手术治疗后复发率可达9%～61%,而肾移植患者在接受患有IgA肾病患者的肾脏捐献移     

成人单纯性血尿IgA肾病临床病理及相关危险因素分析

<正>IgA肾病(IgA nephropathy,IgAN)是常见的原发性肾小球疾病,预后不容乐观[1],临床以反复发作的肉眼血尿或镜下血尿为主要表现,可伴有少量蛋白尿,也可出现肾病综合征。目前关于不伴蛋白尿的单纯性血尿成人IgAN患者临床病理特点及血压、尿酸、血脂等相关危险因素对其的影响报道较少。了解这部分患者的病例特点,有助于更好地指导临床治疗,改善预后。     

蛋白尿致肾小管间质纤维化的机制及防治

肾间质纤维化是各种不同病因的慢性肾脏病进展到终末期肾病（ESRD）的共同病变过程。动物实验和临床试验表明，各种肾脏疾病进行性肾功能恶化主要取决于肾间质损伤的严重程度。蛋白尿是肾小球疾病的共同临床表现，长期蛋白尿不仅引起肾小球硬化，而且可以直接导致肾小管间质损伤，后者与肾小球疾病进展的关系更为密切。我们的研究发现，在血压、肾功能均正常且其他各项临床指标相近的条件下，显著蛋白尿IgA肾病患者的肾小球及肾小管间质损害程度更为严重，蛋白尿可作为独立的致病因子，直接造成IgA肾病患者肾小管间质损害。因此，研究肾间质纤维化的分子机制，探索有效的防治措施，对延缓ESRD的进程意义重大。本文重点阐述蛋白尿致肾小管间质损伤的机制及防治现况。     

IgA肾病蛋白尿的研究进展

IgA肾病是我国慢性肾功能衰竭的主要原发性病因,蛋白尿是 IgA肾病进展和预后的独立危险因素,临床表现为少量蛋白尿和微量白蛋白尿的IgA肾病病理损伤并不轻,需要早期干预治疗。本文就IgA肾病蛋白尿的研究进展作一综述。     

Treatment of IgA nephropathy

IgA nephropathy (IgAN) is an important cause of progressive kidney disease with 25-30% of patients developing end-stage renal disease within 20 years of diagnosis. There is still no treatment to modify mesangial IgA deposition and available treatments are those extrapolated from the management of other patterns of chronic glomerulonephritis. There remains no consensus on the use of immunosuppressive agents for treatment of progressive IgAN and this is compounded by the relative lack in IgAN of randomized controlled trials relevant to current clinical practice. Patients with recurrent macroscopic hematuria or isolated microscopic hematuria and proteinuria <1 g/24 h require no specific treatment. Those with nephrotic syndrome and minimal change on renal biopsy should be managed as for minimal change nephropathy. There is no evidence to support the use of corticosteroids for nephrotic IgAN outside this group of patients. Patients presenting with acute renal failure require evaluation to distinguish acute tubular necrosis, which requires supportive therapy only, from crescentic IgAN, for which treatment with cyclophosphamide and corticosteroids in a regimen similar to that for renal small vessel vasculitis is indicated in the absence of significant chronic histologic injury. Patients at greatest risk of progressive renal impairment are those with hypertension, proteinuria >1 g/24 h, and reduced glomerular filtration rate at diagnosis. All such patients should be treated to a blood pressure of 125/75 mm Hg with dual blockade of the renin-angiotensin system with angiotensin-converting enzyme inhibition and angiotensin receptor blockade. At present, there is insufficient evidence for the additional use of immunosuppressive agents, antiplatelet agents, or anticoagulants.     

Efficacy of tonsillectomy for patients with recurrence of IgA nephropathy after kidney transplantation

Abstract:  From January 2007, we started to perform the tonsillectomy for every patient with recurrent IgA nephropathy (IgAN) after kidney transplantation. Up to September 2008, four recipients with primary IgAN had biopsy-proven recurrent IgAN. They had also progressive hematuria or proteinuria from on the average 14.3 months after transplantation. Then their specimens diagnosed as recurrent IgAN were collected and they underwent tonsillectomies on the average 52.3 months after transplantation. Abnormal urinary findings of all patients favorably improved after tonsillectomy. All cases but one had mild renal injury, where the severity of glomerular lesions, glomerular hypercellularity, segmental lesions, and sclerosis was mild, and no deteriorated serum creatinine (SCr) before their tonsillectomies. Even the case with exacerbated SCr and severe renal injury, where the severity of glomerular lesions was severe, had her urinary findings ameliorated promptly after tonsillectomy likely as others. At present, they have almost no symptoms after tonsillectomy and no remarkable change of SCr level compared with before and after tonsillectomy and maintain ameliorated urinary findings continuously. Tonsillectomy had possibility to be a favorable treatment of hematuria or proteinuria in recurrent IgAN recipients.     

Immunoglobulin A nephropathy complicating ulcerative colitis

Ulcerative colitis is rarely associated with immunoglobulin A nephropathy (IgAN). The development of IgA nephropathy complicates further the clinical course of patients with ulcerative colitis. A 72-year old man with a 30-year history of ulcerative colitis requiring colectomy and modest renal insufficiency secondary to complications of nephrolithiasis and renal artery stenosis developed glomerular hematuria, proteinuria and progressive renal failure. Percutaneous kidney biopsy revealed IgAN with extensive glomerular and interstitial sclerotic changes. After resection of a chronically infected ileo-rectal pouch, renal function improved, while hematuria and proteinuria gradually disappeared without specific treatment of the IgAN. The manifestations of IgAN complicating ulcerative colitis can be improved with effective treatment of the bowel disease even when there are extensive sclerotic changes in the kidneys.     

IgA nephropathy: clinical features and natural history--a pediatric perspective

This review describes the spectrum of clinical features observed in pediatric patients with IgA nephropathy (IgAN) in different parts of the world. The typical clinical presentation consists of an episode of macroscopic hematuria within 24 to 48 hours of an upper respiratory infection. However, many children who present with macroscopic hematuria are subjected to a battery of urologic studies before the appropriate procedure is performed. This sequence highlights the lack of awareness of IgAN among pediatricians, family practitioners, and urologists. The finding of microscopic hematuria or, less commonly, proteinuria, in a urinalysis carried out as part of a school screening program is the most frequent "presentation" of IgAN in Japanese children. However, it is possible that many children with IgAN expressed as microscopic hematuria and/or mild proteinuria remain undiagnosed in this country because routine urinalysis is not done and many pediatric nephrologists are reluctant to perform renal biopsies when such children are identified. It is now recognized that some patients with IgAN and nephrotic range proteinuria exhibit a state of steroid responsiveness. The renal biopsy in such patients often reveals "minimal change." Several recent studies have shown progressive deterioration in approximately 10% of all pediatric patients found to have IgAN and in 15% to 30% of the subset of patients with more severe histologic findings. Hypertension and proteinuria are observed frequently in patients who progress to chronic renal failure. It is proposed that multicenter collaborative studies be designed to evaluate proposed therapies for children with IgAN associated with proliferative glomerular lesions, particularly those in whom hypertension, proteinuria, and depressed glomerular filtration rate are found.     

Combined glomerular deposition of polymeric rat IgA and IgG aggravates renal inflammation

BACKGROUND: IgA nephropathy (IgAN) is characterized by deposition in the glomerular mesangium of IgA together with C3, C5b-9, and properdin. IgG deposition as a risk factor in IgAN was recently confirmed by a long-term follow-up of patients with IgAN. We previously reported on an acute model of IgA-mediated glomerular inflammation in Wistar rats. METHODS: To investigate the effect of the combination of IgA and IgG on glomerular injury, Wistar rats were injected with a minimum dose of rat IgG in the presence or absence of a subnephritogenic dose of polymeric rat IgA. Subsequently, glomerular complement activation, influx of inflammatory cells, proteinuria, and hematuria were assessed. RESULTS: Administration of IgG to the rats resulted in maximal proteinuria of 20.3 +/- 12.1 mg/24 h on day 2 and an absence of overt glomerular inflammation. Administration of polymeric rat IgA antibodies to rats resulted in hematuria with a moderate mesangial complement deposition. In the combination group, however, glomerular deposition of C5b-9 was dramatically increased. This was accompanied by increased proteinuria as compared with rats receiving IgA or IgG antibody injections alone on day 7. Microhematuria occurred in rats receiving either polymeric rat IgA or IgG alone or the combination. While both rat IgG and polymeric IgA induced minor mesangial cell (MC) proliferation and MC lysis, the combination resulted in a pronounced, significant increased percentage of aneurysm formation on day 7 after injection. CONCLUSIONS: We conclude that in this model of IgA-induced glomerulopathy, a selective, complement-dependent glomerular inflammation is induced in Wistar rats by glomerular codeposition of rat isotypic monoclonal antibodies.     

Acute poststreptococcal glomerulonephritis superimposed on IgA nephropathy

Superimposition of poststreptococcal glomerulonephritis (PSGN) on the course of IgA nephropathy (IgAN) is uncommon. A case of PSGN during IgA nephropathy is presented. A 30-year-old man who had alternating gross and microscopic hematuria for 7 months underwent a renal biopsy. The first renal biopsy revealed IgAN with mesangial deposits of IgA and C3. Two months later, the patient suffered generalized edema, proteinuria, hematuria, an increased ASO titer and a decreased C3 level. A second renal biopsy revealed diffuse endocapillary proliferative glomerulonephritis with epimembranous hump-like electron-dense deposits of C3, but the original mesangial IgA deposits had disappeared. A diagnosis of acute PSGN was indicated. Two months after the onset of acute nephritic syndrome, the patient remained asymptomatic, except for microscopic hematuria and proteinuria. Some cases with persistent proteinuria or hematuria after PSGN are probably related to preexisting IgAN.     

The product of duration and amount of proteinuria (proteinuria index) is a possible marker for glomerular and tubulointerstitial damage in IgA nephropathy

BACKGROUND/AIMS: IgA nephropathy (IgAN) is one of the major causes for chronic renal failure (CRF). Presence of massive proteinuria, hypertension, increased serum creatinine level and sclerotic histopathological changes of the glomerulus are known to be determinants for the progression of CRF. However, the relationships between duration of proteinuria/hematuria and histopathological changes, which may be correlated with the renal prognosis, have not been clarified. METHODS: A cross-sectional, univariate analysis of clinical parameters on the four glomerular and three tubulointerstitial histopathological grades in 57 untreated biopsy-proven IgAN patients (M/F = 32/25) was performed. RESULTS: The age at the time of renal biopsy (35.2 +/- 13.0 years; mean +/- SD), average duration of proteinuria (5.3 +/- 5.8 years), mean urinary protein excretion (0.99 +/- 1.22 g/day), serum creatinine (Cr 0.97 +/- 0.28 mg/dl), Cr clearance (Ccr 75.5 +/- 29.4 ml/min), and blood urea nitrogen (BUN 15.4 +/- 3.9 mg/dl) were well correlated with both histopathological grades. The product of duration (years) and urinary protein excretion (g/day) at the time of renal biopsy was more significantly correlated with glomerular and tubulointerstitial histopathological grades and serum Cr. CONCLUSION: The natural course of IgAN is steadily progressive depending on the duration and amount of proteinuria. The product of these two factors (proteinuria index) may be a useful predictor for glomerular and interstitial histopathological changes and the fate of renal function in IgAN.     

Clinicopathological correlations in nephrotic children with IgA nephropathy

Background. The prognostic significance of nephrotic syndrome (NS) in children with IgA nephropathy (IgAN) is unclear. Methods. NS was found in eight children with IgAN (mean onset age, 9.3 years). The clinicopathological findings of these eight children were investigated. Results. Five patients presented with macroscopic hematuria, while the remaining three were discovered in a school urinary screening program or by chance urinalysis. Six patients developed NS at the onset, and two developed NS later in the course of IgAN. All patients were treated with corticosteroids. At the end of follow-up, heavy proteinuria persisted in four children, one of whom had renal dysfunction at the onset of NS and developed end-stage renal failure, and two of whom developed NS after the onset of IgAN. Proteinuria decreased to less than 1 g/day 3 months after NS in four patients, two of whom showed disappearance of proteinuria afterward. Renal biopsy specimens revealed mesangial proliferation and crescent formation in all patients. The degree of persisting proteinuria was correlated with the presence of glomerular sclerosis, fibrous crescents, tubulo-interstitial changes on light microscopy, and depositions of C3 on immunofluorescence microscopy. Conclusions. Children who developed NS after the onset of IgAN developed renal dysfunction; the prognosis of those who showed chronic histopathological changes on renal biopsy specimens was poor, even in these young children. Received: April 17, 2000 / Accepted: July 4, 2000     

IgA肾病诊疗进展

IgA肾病是全球最常见的原发性肾小球肾炎,亚洲人群中发病率高于其他人种。IgA肾病是目前导致终末期肾病的重要原因之一。临床上以血尿为特点,常伴随蛋白尿、高血压。其病理表现主要为IgA免疫复合物在肾小球系膜区的沉积、系膜细胞增生、毛细血管内皮细胞增生等。其发病机制可能为血液循环中半乳糖缺乏的IgA1增多,在內外界环境刺激下,产生过多的、能沉积于肾小球系膜区的免疫复合物。目前,对IgA肾病的诊断主要依靠病理检查。治疗方面,以肾素-血管紧张素系统阻断剂、控制血压为基础,恰当联合免疫抑制剂、细胞毒性药物、鱼油等或能延缓IgA肾病的进展。本文的目的是对IgA肾病的诊疗现状进行总结和分析,为临床工作及进一步科研提供指导和参考。     

IgA肾病520例临床病理分析

目的研究IgA肾病(IgAN)的临床和病理特点及其相互关系。方法对1992年11月～2003年6月温州医学院附属第一医院肾内科病理室肾活检诊断的原发性IgAN520例进行临床与病理分型关系的分析。结果520例IgAN临床表现以无症状性尿检异常最常见,占346例(66.5%),其次是慢性肾炎和肾病综合征,分别占77例(14.8%)和66例(12.7%)。病理类型以局灶节段硬化性肾小球肾炎最常见,占186例(35.8%),其次是系膜增生性肾小球肾炎、轻微病变肾小球肾炎和局灶节段增生性肾小球肾炎,分别为116例(22.3%)、104例(20%)和63例(12.1%)。结论IgAN的临床病理表现多样化并具有一定特点。临床表现最常见为无症状性尿检异常,在病理上最常见的是局灶性肾小球病变类型。