儿童IgA肾病的临床与病理分析

目的 探讨IgA肾病患儿临床表现和病理改变的关系及疾病转归.方法 分析30例IgA肾病患儿的临床和病理改变,病理分型采用改良Lee分型法,其中28例进行随访,4例重复肾活检.结果 临床表现孤立性血尿型10例,病理为Ⅰ、Ⅱ级;血尿和蛋白尿型11例,病理为Ⅰ～Ⅲ级;急性肾炎型2例,病理Ⅲ级;肾病综合征型7例,病理为Ⅱ～Ⅳ级,以Ⅱ、Ⅲ级为主.4例行重复肾活检,病理变化为改善、加重和无明显变化,但免疫复合物沉积均有加重.28例随访1年2个月～9年,其中9例预后不乐观;死亡1例;预后不良患儿7例治疗依从性差.结论 IgA肾病临床表现与病理有一定相关性,急性肾炎型、肾病综合征型患儿损伤重、预后欠佳,同时提示临床医师重视IgA肾病患儿随访和健康宣教,提高治疗依从性,以利疾病康复.     

IgA肾病合并急性肾衰竭的临床与病理分析

目的：探讨IgA肾病合并急性肾衰竭（ARF）的临床与病理特点。方法：1992年～2006年经肾活检确诊IgA肾病合并ARF 20例患者的临床与病理资料进行回顾性分析。根据不同病理选择治疗方案并进行随访。结果：本组20例IgA肾病合并ARF,占活检IgA肾病的3.8%（20/527）。其中急性肾炎综合征4例,急性肾炎综合征合并肾病综合征5例,以浮肿、少尿为主8例,以恶性高血压为主3例。病理改变上系膜增生性肾炎5例,新月体肾炎8例,增生硬化性肾炎伴新月体肾炎3例、轻度系膜增生性肾炎合并急性肾小管间质肾炎4例。14例肾功能恢复正常,4例部分缓解,2例无效性透析治疗后行肾移植。结论：IgA肾病合并急性肾衰竭发生率达3.8%,高于目前文献报道。临床表现多样化,病理表现为多种病理类型。病理轻则预后好,新月体肾炎诊断治疗及时预后好,多数患者肾功能可以恢复正常。因此早期及时肾活检对IgA肾病指导治疗、判断预后有非常重要的价值。     

移植肾IgA肾病三例报告及文献复习

目的 探讨移植肾IgA肾病的病理特征、临床表现及预后。方法 回顾分析移植肾穿刺活检病理及临床资料。结果 3例IgA肾病,1例为复发性膜增殖型,2例为复发／发性系膜增殖型,临床表现主要是镜下血尿、轻度蛋白尿、或／和高血压、高脂血症;病理改变与普通人群的IgA肾病相同,此外存在多灶性肾小管萎缩及间质纤维化;其对治疗的反应与病理改变密切相关,以双嘧达莫、小剂量华法林及雷公藤多甙治疗2年,2例系膜增殖型者肾功能稳定,膜增殖型的1例治疗2年半后血肌酐上升。结论 移植肾IgA肾病复发迅速,其进展速度及治疗效果与病理分型和病变程度密切相关,无特效治疗方法。     

IgA肾病患者尿蛋白波动程度与病理分级的相关分析

IgA肾病是一组不伴有系统性疾病，肾活检免疫病理检查在肾小球系膜区以IgA为主的团块样或颗粒样沉积，临床上以血尿、蛋白尿为主要临床表现的。肾小球肾炎，其病因和发病机制未完全明确。有1／3的IgA肾病患者可在10～20年内发展为慢性肾衰竭，甚至相当一部分在5年内发展至尿毒症期。肾病理改变是判断预后的重要依据，病理分级越高预后越差。蛋白尿是IgA肾病患者常见的临床表现，以往的大量研究表明蛋白尿与疾病病理改变的严重程度相关，并作为影响疾病预后的独立因素。但是，尿蛋白的波动性与病理改变之间的相互关系鲜有研究与报道。我们对158例IgA肾病患者的尿蛋白的波动程度与其病理改变的相关性进行了研究，以期提高临床医生分析病理及判断预后的能力。     

肾病综合征表现的儿童IgA肾病临床和病理分析

目的:分析儿童IgA肾病(IgA nephropathy,IgAN)中表现为肾病综合征的患儿临床和病理的特点.方法:总结1995年12月～2004年6月我科98例肾活检病理诊断为原发IgAN中,表现为肾病综合征(NS)的临床、组织病理特点.结果:98例中表现为肾病综合征22例,占IgAN的(22.4%).按1982年WHO病理组织分类,肾小球病理改变为Ⅰ级2例(9.1%),1例单纯性肾病,1例肾炎性肾病;Ⅱ级5例(22.7%),1例单纯性肾病,4例肾炎性肾病;Ⅲ级9例(40.9%),Ⅳ级6例(27.3%),均有肾炎性肾病.免疫荧光分型:IgA型5例(22.7%),IgA IgG型4例(18.2%),IgA IgM型9例(40.9%),IgA IgG IgM型4例(18.2%),以IgA IgM型多见.小管间质损害(TIL)分型:Ⅰ级11例(50.0%),Ⅱ级7例(31.8%),Ⅲ级4例(18.2%),Ⅳ级未见.结论:IgA肾病表现为肾病综合征者临床及病理改变均较重,肾小球变化以Ⅲ～Ⅳ级改变为主,免疫荧光中以IgA IgM型多见,小管间质损害以Ⅰ级和Ⅱ级多见.     

120例IgA肾病临床表现与病理特点分析

目的：探讨IgA肾病患者临床表现、病理特点及其相关性。方法：回顾性总结我院肾内科2004年1月-2005年12月期间肾活检为原发性IgA肾病的临床和病理资料。结果：120例IgA肾病患者发病平均年龄为35岁,男女比例为3∶4,占我院同期肾活检的38.46%。有诱因发者40例（33.33%）,其中上呼吸道感染22例（55%）。临床表现多样化,以蛋白尿＋血尿型最常见,共60例（50%）,该型病理改变程度较重,Ⅲ级及Ⅳ级均占该型的43.3%。肾衰竭型18例（15%）,该型多伴高血压及较多量的蛋白尿（〉1.5g/24h）,病理分级多为Ⅳ-Ⅴ级。肾病综合征型为14例（11.67%）,病理分级Ⅳ-Ⅴ级者12例。单纯血尿型17例（14.17%）,病理分级以Ⅲ级（47.06%）多见。单纯蛋白尿型11例（9.17%）,病理分级亦以Ⅲ级（63.63%）多见。结论：IgA肾病发病率较高,大多在感染诱因下发作或加重。临床表现多样化,其中蛋白尿＋血尿型为最常见的临床表现。肾衰竭型（多伴有高血压、大量蛋白尿）的发病比例有增高趋势,该型预后不良,临床应根据病理结果积极治疗,以延缓肾衰竭进展。     

IgA肾病小管间质损害与预后相关分析

目的观察IgA肾病患者肾小管间质损害与临床表现及肾组织病理改变之间的关系。方法选取经临床病理诊断明确的IgA肾病患者143例,根据小管间质损害的程度进行临床和病理指标的比较。结果①143例IgA肾病患者中,83．92％出现小管间质损害,其中轻度占65．03％,中度占16．08％,重度占2．80％。②随着小管间质损害程度的加重,女性患者所占比例逐渐增大,年龄和血压呈逐渐升高的趋势,血浆白蛋白水平呈下降趋势,而24h尿蛋白定量、血肌酐、总胆固醇水平呈明显升高趋势。③随着小管间质损害的加重,患者肾小球损害和血管损害也随之加重（P〈0．05）。结论小管间质损害是决定IgA肾病预后的关键因素之一,但不是唯一因素,应将临床参数与病理参数,间质损害指标与肾小球、肾血管损害指标相结合,综合评价患者预后。     

表现为孤立性血尿的IgA肾病的病理特征和转归

目的探讨表现为孤立性血尿的IgA肾病病理特征及其与预后关系。方法分析103例经肾活检确诊的呈孤立性血尿IgA肾病患者的临床和病理特征,并观察其转归。以Ser升高一倍为终点,用Kaplan-Meier生存曲线分析肾脏无事件存活率。结果呈孤立性血尿的lgA肾病Lee病理分级分布以Ⅱ-Ⅲ级为主,但有20.4％为LeeⅣ-Ⅴ级和12.7％有肾间质纤维化。经过(49.5±35.4)月的随访,Set水平从(73±16)μmol/L上升至(96±114)μmol/L,但差异无统计学意义(P>0.05)。患者3、5和10年肾存活率分别为100％、100％和85.7％。结论呈孤立性血尿的IgA肾病病理改变轻,中期预后好,但应注意临床与病理不符要并长期随访、监测肾功能进展的指标。     

IgA肾病湿热证与肾穿刺活检病理组织关系的临床研究

目的：研究IgA肾病湿热证与肾穿刺活检病理组织的关系。方法：每例肾穿刺活检病理组织均经光镜，酶标检查。结果：全病程的湿热总分与肾小球病变度、小管间质病变度、系膜增生度、免疫沉积度呈显或非常显等级相关；肾穿时的湿热证分值与肾小球病变度、系膜增生度、免疫沉积度无显相关。结论：(1)IgA肾病中，湿热影响IgA肾病的病理过程，加重肾脏的损害，进而影响预后及转归。(2)肾小管间质损害与湿热证的发生同步。湿热证时肾脏细胞因子和炎症因子活跃造成了小管和间质的急性损伤。(3)肾小球的损害与湿热证不同步，有时间上的相对滞后，反映了免疫复合物的产生和在肾脏局部的堆积而引起肾小球损害需要一定的时间。     

轻度蛋白尿IgA肾病的临床研究进展

正IgA肾病是以IgA或IgA为主的免疫球蛋白沉积在肾小球系膜区及毛细血管袢为特征的原发性肾小球疾病,其临床表现和病理变现呈多样性。蛋白尿水平作为一重要临床指标被认为与IgA肾病长期预后息息相关。较多蛋白尿往往提示肾脏病理损伤严重,需要积极干预,且多数预后不良。而对于轻度蛋白尿的IgA肾病患者的评估目前尚无明确的定论。本文就表现为轻度蛋白尿(1.0 g/d)的IgA肾病患者从临床病理、     

Repeat renal biopsy in children with IgA nephropathy

Serial renal biopsy findings in 61 children with IgA nephropathy were correlated with their clinical course. At the time of the second biopsy, 23 patients showed clinical remission defined as complete disappearance of proteinuria and hematuria with normal renal function while 38 had persistent urinary abnormalities with normal renal function at the second biopsy. There were no differences between the two groups with regard to initial clinical findings and pathologic findings of the initial renal biopsy. The second biopsy of patients with clinical remission showed improvement of the glomerular changes on light microscopy, disappearance or diminution of IgA deposits in the mesangium and decrease of electron-dense deposits, whereas the second biopsy of patients with persistent urinary abnormalities showed progression of glomerular changes on light microscopy, persistence of mesangial IgA deposits and persistence of electron-dense deposits. Our study results show the importance of repeat renal biopsy in children with IgA nephropathy with persistent urinary abnormalities, as a progression of glomerular changes is common in these patients. These observations suggest that the deposition of IgA in the mesangium may be responsible for the glomerular damage in children with IgA nephropathy.     

妊娠时肾脏损害临床和肾组织病理变化的分析研究

目的：为探讨妊娠致肾脏损害的特点及病理特征。方法；对52例妊娠致肾脏损害住院病人进行了临床和肾活检病理的分析研究。结果：52例终止妊娠3个月后仍有蛋白尿，合并肾功能不全者28例（53．85％），肾活检免疫病不示IgG34例，IgA38例，IgM28例及C340例肾小球血管袢及系膜区沉积；病理表现系膜增生性肾炎34例、膜增生性肾炎10例，局灶节段性肾小球硬化6例，膜性肾病2例，52例中合并小球硬化者30列（57．69％）；胎儿死亡40例（75．47％）。结论：妊娠激发了免疫反应导致肾脏病变是肾小球疾病发病机制之一，积极诊治该类病人是临床不可忽视的问题。     

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.     

Clinical and pathological characteristics and outcomes of Chinese patients with primary anti-neutrophil cytoplasmic antibodies-associated systemic vasculitis with immune complex deposition in kidney

AIM: To analyse the clinical and pathological characteristics of Chinese patients with immune complex deposition in kidney in anti-neutrophil cytoplasmic antibodies (ANCA)-positive vasculitis. METHODS: Enrolled in this study are patients with immune complex deposition in kidney in ANCA-positive vasculitis diagnosed in Peking University First Hospital. Their clinical and pathological data were collected and analysed. RESULTS: Twenty-three patients were eligible. Fifteen patients were with microscopic polyangiitis and eight patients were with Wegener's granulomatosis. The mean age was 48.8 years and with a male/female ratio of 10/13. The interval, between onset of disease and the diagnosis of disease, was 429.6 +/- 693.3 days. All patients had clinical evidence of renal involvement. The major immunoglobulin deposited was IgM and the main locations were mesangial and sub-epithelial area. Four patients also presented features of membranous nephropathy and six patients presented features of IgA nephropathy. About 52.2% of patients had hypocomplementaemia. All patients received immunosuppressive therapy and all of them achieved clinical remission. Patients were followed for about 28.8 +/- 25.3 months. Nine patients kept clinical remission, nine patients progressed to end-stage renal disease and five patients died. When these patients are compared with patients who had classical pauci-immune vasculitis, they had greater proteinuria (P < 0.05), higher prevalence of hypocomplementaemia (P < 0.05) and greater glomerular cellularity (P < 0.05). CONCLUSION: The present study showed that the features of patients with ANCA-associated vasculitis with immune complex deposition in kidney were similar with classical 'pauci-immune' vasculitis except for more proteinuria, more hypocomplementaemia and greater glomerular hypercellularity.     

Case of rheumatoid arthritis with various histological lesions of the kidney

We experienced a case of rheumatoid arthritis with nephrotic syndrome. A renal biopsy specimen from this patient showed various renal histological changes. The patient was a 50-year-old man who was diagnosed as having rheumatoid arthritis in 1987. We performed a renal biopsy because he had persistent proteinuria from March in 2002. The renal biopsy specimen showed amyloid AA and P protein deposition in the glomeruli. Moreover mild mesangial proliferation was recognized. IgA-deposition in the mesangial area, and granular-deposition of IgG along the glomerular capillary wall were also observed. In electron microscopy, electron dense deposits were recognized in the mesangial area and subepithelium of the glomerular basement membrane. From these findings, we diagnosed amyloid nephropathy, IgA nephritis and membranous nephropathy. Renal biopsy of patients with RA is useful not only for precise diagnosis, but also for selection of the appropriate treatment.     

Pathology, clinical presentations, and outcomes of C1q nephropathy

C1q nephropathy is an uncommon glomerular disease with characteristic features on immunofluorescence microscopy. In this report, clinicopathologic correlations and outcomes are presented for 72 patients with C1q nephropathy. The study comprised 82 kidney biopsies from 28 children and 54 adults with male preponderance (68%). Immunofluorescence microscopy showed dominant or co-dominant staining for C1q in the mesangium and occasional glomerular capillary walls. Electron-dense deposits were observed in 48 of 53 cases. Light microscopy revealed no lesions (n = 27), focal segmental glomerulosclerosis (FSGS; n = 11), proliferative glomerulonephritis (n = 20), or various other lesions (n = 14). Clinical presentations in the patients who had no lesions histology were normal urine examination (7%), asymptomatic hematuria and/or proteinuria (22%), and nephrotic syndrome (minimal change-like lesion; 63%), which frequently relapsed. All patients with FSGS presented with nephrotic syndrome. Those with proliferative glomerulonephritis usually presented with chronic kidney disease (75%) or asymptomatic urine abnormalities (20%). Of the patients with sufficient follow-up data, complete remission of the nephrotic syndrome occurred in 77% of those with a minimal change-like lesion, progression to end-stage renal disease occurred in 33% of those with FSGS, and renal disease remained stable in 57% of those with proliferative glomerulonephritis. In conclusion, this study identified two predominant clinicopathologic subsets of C1q nephropathy: (1) Podocytopathy with a minimal change-like lesion or FSGS, which typically presents with nephrotic syndrome, and (2) a typical immune complex-mediated glomerular disease that varies from no glomerular lesions to diverse forms of glomerular proliferation, which typically presents as chronic kidney disease. Clinical presentation, histology, outcomes, and presumably pathogenesis of C1q nephropathy are heterogeneous.     

Efficiency of glucocorticoid treatment in IgA nephropathy with massive proteinuria

Objective To investigate the clinicopathological characteristics of IgAN patients with massive proteinuria, as well as their treatment response to glucocorticoids and long-term prognosis. Methods Clinical and pathological parameters were collected in patients diagnosed with IgA nephropathy in our hospital from Jan 2003 to Oct 2015. Patients were followed up for at least six months under the treatment with full dosage of glucocorticoids. Responses of patients with and without nephrotic syndrome were compared. Results A total of 156 patients were enrolled for the analysis (86 patients in the nephropathic proteinuria group, and 70 patients in the nephrotic syndrome group). Patients presented with nephrotic syndrome showed higher proportion of IgM deposition in renal slides. There exited no difference in treatment response to glucocorticoids between the two groups. Patients with full or partial remission showed a better prognosis by Kaplan-Meier analysis than no remission group (P＜0.001). The ratio of segmental sclerosis was negatively correlated with treatment response to glucocorticoids by multiple linear regression (β value=-0.330, P＜0.001). Multivariate Cox regression model showed that glomerular density (HR=0.45, P=0.02) and eGFR (HR=0.95, P=0.001) were independent influential factors for renal survival. Conclusions Patients presented with nephrotic syndrome show higher proportion of IgM deposition in renal slides. Patients in remission after treatment with 6-month glucocorticoids present a better prognosis than no remission patients, and glomerular density as well as eGFR are independent influential factors for renal survival.     

The spectrum of acute renal failure in IgA nephropathy

Background: Acute renal failure rarely complicates the course of IgA nephropathy. In this study, we have tried to define the mode of presentation, the spectrum of morphology, and the prognostic factors for renal outcome. Methods: Twenty patients with biopsy-proven IgA nephropathy who developed acute renal failure were identified from 2000 to 2009 at a medical center in Taiwan. The patients' records were retrospectively reviewed with respect to clinical presentation, morphology of renal biopsy, and outcomes. Results: On histology, glomerular crescents were present in 11 patients (55%), acute tubular necrosis was identified in 11 patients (55%), acute interstitial nephritis was seen in 4 patients (20%), and extensive tubular red blood cell casts were present in 4 patients (20%). At the end of follow-up, 2 patients (10%) had died, 11 patients (55%) were in remission, and 7 patients (35%) developed end-stage renal disease. The prognostic factors for renal outcome were peak serum creatinine, dialysis support requirement, morphology (prominent glomerular/tubular injury), percentage of glomeruli affected by crescents, and interstitial infiltration (p = 0.04, <0.001, 0.013, 0.05, 0.02, respectively). Conclusions: Our findings suggested that there were four pathogenic mechanisms involved in IgA nephropathy with acute renal failure including (1) crescentic IgA nephropathy; (2) acute tubular necrosis associated with microhematuria and red blood cell casts occluding tubules; (3) acute tubular necrosis not related to microhematuria; and (4) acute interstitial nephritis, apparently induced by drugs. In general, patients with prominent tubular injury had a much higher remission rate than patients with prominent glomerular injury.     

原发性IgA肾病与非IgA系膜增生性肾小球肾炎的临床病理分析

目的 比较原发性IgA肾病与非IgA系膜增生性肾小球肾炎（non-IgA mesangial proliferative glomerulonephritis,non-IgA MsPGN）的临床及肾脏病理改变特点.方法 选择我科经肾活检确诊的原发性IgA肾病患者（A组）和non-IgA MsPGN患者（B组）进行临床与病理资料对比分析.结果 A、B组的性别、前驱上呼吸道感染诱因、起病时伴发高血压、镜下血尿、血肌酐无统计学差异（P＞0.05）.B组较A组起病年龄小,起病时伴发肉眼血尿比率低,肾病综合征发生率高,血IgG水平低,差异均有统计学意义（P＜0.05）.A组肾小球、肾小管间质、肾小动脉病理改变发生率高于B组（P＜0.05）,IgM、C3沉积、系膜区电子致密物沉积、大块状致密物、足细胞微绒毛化、肾小球基底膜分层发生率均较B组高（P＜0.01）.结论 IgA肾病与non-IgA MsPGN在临床表现、病理改变上存在明显差异,IgA肾病较non-IgA MsPGN病理损伤重.     

Differences in new-onset IgA nephropathy between young adults and the elderly

Background: The goal of this study was to define the clinical and histological differences in new-onset IgA nephropathy between young adults and the elderly. Methods: We retrospectively examined renal biopsy findings, clinical features at presentation and outcomes in 82 young adults (mean age 30.3 ± 10.2 years) and 17 elderly patients (mean age 71.9 ± 4.5 years) with IgA nephropathy whose renal biopsies were taken within 1 year from the onset of renal manifestations. Results: The elderly group more frequently had hypertension (p < 0.001), acute renal failure (p < 0.001), and nephrotic range proteinuria (p = 0.001) at presentation than the young adults group. On histology, a higher percentage of globally sclerotic glomeruli (p < 0.001) was present in the elderly group. In patients presenting with acute renal failure, the elderly group more frequently had an intercurrent disease (p = 0.02), mostly infection, and a higher mortality rate (p = 0.033). On histology, the young adults group had a higher percentage of glomeruli affected by crescents (p = 0.027); in contrast, the elderly group more commonly had acute tubular injury (p = 0.02). Conclusions: The elderly patients affected by IgA nephropathy had more severe renal manifestations at presentation (acute renal failure in 52.9% and nephrotic syndrome in 41.2% of patients). In cases of acute renal failure, the elderly patients had more predominant tubular rather than glomerular injury. Moreover, the considerable mortality rate (44.4%) might be associated with the intercurrent disease, mostly infection, which was more commonly present in the elderly patients.