临床及肾脏病理与狼疮性肾炎肾脏损害的相关性

目的:探讨狼疮性肾炎(LN)患者临床及病理与肾脏损害之间的关系,明确肾脏损害的相关血清学指标及病理表现.方法:回顾性分析我院182例LN患者的临床及病理资料,进行统计学分析.结果:(1)LN患者Ⅳ型和Ⅴ型的肾脏损害(蛋白尿、血尿、血肌酐)最重,抗ds-DNA抗体、抗核小体抗体及补体C3降低在Ⅳ型多见.(2)血脂、血浆白蛋白及血红蛋白均与肾脏损害明显相关(P＜0.01).(3)肾脏损害指标升高时,免疫复合物C1q、C3、EM沉积均达90%以上;肾脏损害指标与AI、CI及其病理参数明显正相关.结论:血脂异常、低蛋白血症、贫血、抗ds-DNA抗体、核小体抗体及补体C3均为提示肾脏损害程度的血清学指标.Ⅳ型和Ⅴ型的肾脏损害最重,并且蛋白尿、血尿、血肌酐水平与肾脏的病理沉积相关.     

狼疮肾炎患者肾组织CD134(OX40)表达的免疫组织化学分析

目的：了解狼疮肾炎(LN)肾组织中CD134(OX40)的表达并探讨其与LN肾脏病理改变和肾功能损害的关系。方法：用免疫组织化学方法对40例LN患肾组织CD134的表达进行检测，并对其与LN的肾脏病理改变和肾功能损害的相关性进行分析。结果：LN患肾组织中，CD134表达显上调，尤其以WHO Ⅳ型LN更为明显。除系膜细胞、内皮细胞和远曲小管CD134表达明显增强外，肾间质毛细血管和大血管内皮细胞亦有CD134表达阳性的浸润细胞。LN肾组织加以表达与LN肾脏病理活动指数和肾功能损害显相关。结论：LN患确实存在CD134共刺激分子的异常表达，这在LN的发生和发展中可能起着重要的作用。     

狼疮性肾炎分型分阶段中西医结合治疗

系统性红斑狼疮 (SLE)是我国最常见的风湿病之一 ,发病率约占 70例 /10万人口。SLE伴肾脏损害占 70 %以上 ,而病理检查几乎 10 0 %有肾脏损害。狼疮性肾炎 (LN)占我国继发性肾炎的第一位。LN的治疗近年来取得了重大进展 ,10年存活率已提高到 90 %以上。糖皮质激素及免疫抑制剂仍然是LN的首选药物 ,特别是美国国立卫生研究院(NIH)提倡的大剂量糖皮质激素联合环磷酰胺 (CTX)冲击方案使许多重型LN获得缓解。新的免疫抑制剂如环孢霉素A(CsA)及霉酚酸酯 (MMF)的问世又使部分难治性LN获得缓解。尽管如此 ,仍有许多问题未能解决 ,肾衰…     

核因子-κB在狼疮肾炎肾组织中的表达及其意义

目的了解狼疮肾炎(LN)肾组织中核因子(NP)-κB的表达并探讨其与LN肾脏病理改变和肾功能损害的关系.方法以NF-κB亚基P65单抗为抗体,采用微波免疫组织化学染色(APAAP法)检测LN肾组织NF-κB表达,并进一步分析其与肾小球内C-myc蛋白表达、LN活动指数、肾脏病理和功能损害的关系.结果狼疮肾炎肾组织中NF-κB表达较正常肾组织显著增高,以WHOⅣ型为最显著.NF-κB在肾小球和肾小管均有表达,但小管表达更显著.LN肾组织NF-κB阳性细胞数与肾小球C-myc蛋白表达量、肾组织活动指数、肾脏病理改变和肾功能损害显著相关.结论NF-κB可能参与了LN的发病机制,肾组织中NF-κB的表达可作为反映狼疮肾组织活动病变和进行性肾损害的参考指标.     

儿童狼疮性肾炎的临床特征

目的探讨儿童狼疮性肾炎(LN)的临床特征和实验室检查。方法74例小儿LN和130例成人LN进行临床及血液学、免疫学检查比较。结果表明小儿LN表现为肾病综合征、肾综并肾脏损害、心脏、神经精神和血液系统等损害明显多于成人LN；无症状蛋白尿血尿、关节炎和雷诺征明显低于成人；但免疫学检查与成人无明显差异。结论小儿LN的临床表现及实验室检查有其自身特点。了解小儿LN的临床特征及实验室意义．有助于正确诊断儿童LN。     

尿巨噬细胞移动抑制因子浓度与狼疮肾炎肾组织损害的关系

目的观察狼疮肾炎(LN)患者尿巨噬细胞移动抑制因子(MIF)浓度是否升高及其与LN肾组织MIF表达、肾脏病理及功能损害的关系,试图寻找一种反映狼疮肾损害的非创伤性指标。方法用ELISA方法测定LN患者血、尿MIF浓度。用免疫组织化学双染技术观察肾组织MIF表达及巨噬细胞浸润情况。了解尿MIF水平与狼疮肾组织MIF表达、巨噬细胞浸润、狼疮肾组织活动指数、肾脏功能及组织学损害的关系。结果LN患者肾组织MIF表达及尿MIF浓度较正常人明显增高,尤以增生及炎症明显的Ⅲ、Ⅳ型LN为明显,尿MIF浓度与肾组织MIF表达、巨噬细胞浸润、狼疮肾组织活动指数、肾小管损害显著相关,但与血MIF水平、蛋白尿程度及肾功能损害无显著相关。结论LN患者尿MIF浓度明显增高,并与肾组织MIF表达及狼疮肾组织活动情况显著相关,可作为一种监测狼疮肾活动及肾损害的非创伤性指标。     

中老年狼疮性肾炎的特点及治疗体会

系统性红斑狼疮(SLE)是较常见的结缔组织病，它好发于青年女性，50岁以上的中老年少见。狼疮性肾炎(LN)则是在此基础上合并有肾脏的损害。现将近年来我们收治的630例SLE中的42例中老年LN的特点及其治疗作一探讨。     

陈以平教授治疗狼疮性肾炎的经验

狼疮性肾炎(LN),是一种包括肾脏在内的多个脏器受累的炎症性自身免疫性疾病。临床上以发热、关节炎、皮疹及肾脏损害症状为主要表现。中医学文献中无LN的病名记载。根据LN主要临床特征,可隶属于中医发热、红蝴蝶、日晒疮、水     

血管内皮细胞生长因子表达与狼疮肾炎肾脏病理的关系

研究发现血管内皮细胞生长因子(VEGF)有增加血管通透性、促进内皮细胞增殖、血管生成修复等功能。狼疮肾炎(LN)肾脏病理改变呈多样化,并多伴有肾脏微血管或小血管的损害。VEGF在LN的表达如何并在疾病转归上是否有一定作用,目前研究不多。我们对LN患者肾组织VEGF的表达进行了研究。一、对象与方法1.病例选择:LN患者40例,为1999年1月～2003年4月我院住院患者,其中男6例,女34例,平均年龄(30.6±12)岁。全部病例符合美国风湿病协会1982年系统性红斑狼疮(SLE)诊断标准,并有持续性蛋白尿超过0.5g/d或(和)管型尿,确诊为LN。按世界卫生组…     

血清部分细胞外基质指标检测在早期肾脏纤维化诊断中的意义

本研究评估血清透明质酸(HA)、层粘连蛋白(LN)、Ⅲ型前胶原(PCⅢ)、Ⅳ型胶原(Col Ⅳ)、组织基质金属蛋白酶1抑制剂(TIMP-1)在早期肾脏纤维化诊断中的价值.     

68例狼疮肾炎的肾小管间质病变探讨

目的探讨肾小管间质病变（ＴＩＬ）在狼疮肾炎（ＬＮ）的意义。方法分析了６８例ＬＮ资料，并对其中４２例作了较长期的随访。结果ＬＮ的ＴＩＬ发生率为７５％，ＴＩＬ明显者，肾小管功能显著下降，血肌酐、尿蛋白水平明显增加（Ｐ＜００１），肾小管间质与肾小球急、慢性病理改变之间均显著相关（Ｐ＜００１），同时，存在明显ＴＩＬ者，其生存率及肾存活率有下降趋势。结论ＴＩＬ与肾小球病变呈平行的正相关关系，且对ＬＮ预后有一定的影响。     

激素联合来氟米特治疗狼疮性肾炎疗效的观察

目的 了解来氟米特治疗Ⅳ型狼疮性肾炎的临床效果、安全性和不良反应.方法 59例狼疮性肾炎患者的肾活组织检查(简称活检)显示为狼疮性肾炎,应用来氟米特联合糖皮质激素治疗6个月.用药期间监测血、尿常规、24 h尿蛋白定量、肝、肾功能、抗核抗体及抗双链抗体滴度、红细胞沉降率和补体C3等,6个月后行疗效和安全性的评价.结果 来氟米特治疗狼疮性肾炎尿蛋白缓解率为72.4%,高于环磷酰胺冲击治疗缓解率(57%),狼疮性肾炎活动性指标缓解率也高于后者.结论 来氟米特联合糖皮质激素治疗能有效地控制狼疮活动且不良反应轻,耐受性好.     

Clinicopathology of childhood-onset renal systemic lupus erythematosus

AIMS: To determine the clinicolaboratory renal manifestations; glomerular, extra-glomerular histopathologic lesions; renal tubular dysfunction (RTD) frequency and outcome of a short-term renal follow up in Nigerian children with systemic lupus erythematosus (SLE). METHODS: A non-randomized prospective study of consecutive cases of childhood-onset SLE with nephropathy was conducted. Baseline/follow-up clinicolaboratory data were collected. Each patient was followed up for 12 months. RESULTS: Seven of the 11 children studied were girls. The median age at diagnosis was 11.0 years. Median diagnosis time interval (1.9 years) and median time of renal disease onset (1.0 year) were similar. Hypertension, nephrotic syndrome and acute renal failure (ARF) occurred in 45.5%, 54.5% and 63.7% of the patients, respectively. The glomerular lesions were non-proliferative lupus nephritis (LN) in 9.0% (class II LN); focal (class III LN) and diffuse (class IV LN) proliferative LN (PLN) in 27.0% and 64.0%, respectively. Tubulointerstitial nephritis (TIN, 91.0%) and RTD (64.0%) were common. ARF (P = 0.033) and RTD (P = 0.015) were significantly associated with severe TIN. Complete renal remission rate at end-point was 71.4%. Relapse and renal survival rates were 14.3% and 86.0%, respectively. RTD was persistent in 43.0%. CONCLUSION: Renal function disorders, diffuse PLN and extra-glomerular lesions were frequent. Significant association of ARF and RTD with severe TIN in this series suggests the need for early renal tubular function (RTF) assessment in our SLE patients. Deranged RTF may be marker of severe TIN in SLE warranting early confirmatory renal biopsy and aggressive interventional treatment.     

细胞周期抑制蛋白p21 cipl在狼疮肾炎肾组织中的表达及其意义

目的 了解狼疮肾炎（LN）中细胞周期抑制蛋白p21^cip1的表达并探讨其与肾脏病病理改变，细胞增生及LN肾组织活动性的关系。方法 采用微波免疫组织化学染色法检测LN肾组织p21^cipl表达，并进一步分析其与肾小球增殖细胞核抗原（PCNA）阳性细胞数、LN活指数、细胞增生等肾脏病理改变的相关关系。结果 正常肾小球细胞无或仅有p21^cipl表达，LN肾小球细胞p21^cipl的表达显著上调。尤以非Ⅳ型为显著，LN肾组织p21^cipl阳性细胞数，PCNA阳性细胞数及LN肾组织活动指数显著负相关，结论 p21^cipl在LN肾小球细胞中呈高表达，且与肾小球细胞增生程度密切相关，p21^cipl可能参与LN发病的过程。     

狼疮肾炎终末期狼疮活动情况和预后的观察

目的探讨狼疮肾炎（ＬＮ）患者发展至终末期肾病（ＥＳＲＤ）时狼疮活动情况和预后的关系。方法分析１９８７～１９９７年资料完整的７１例ＬＮ的ＥＳＲＤ患者临床性ＳＬＥ活动和血清学ＳＬＥ活动的各项指标、死亡原因,分析比较ＬＮ的ＥＳＲＤ患者与非ＬＮ的ＥＳＲＤ患者的存活率。结果本组ＬＮ的ＥＳＲＤ患者在进行透析治疗前有６４８％已没有ＳＬＥ临床活动表现。进行透析治疗后,ＳＬＥ临床性活动渐趋静止状态,活动率从第一年５６３％下降至第十年２８％;ＳＬＥ血清学活动表现的消退,要比临床活动表现的消退缓慢;ＬＮ的ＥＳＲＤ透析治疗患者第２、５、１０年的存活率与非ＬＮ透析患者相近,分别为７９６％、５２５％、３０５％,其死亡原因似与ＳＬＥ活动无关。结论在ＬＮ的ＥＳＲＤ期间,ＳＬＥ活动的静止趋势与时俱增,死亡原因与ＳＬＥ活动性似无明显关系,ＬＮ的ＥＳＲＤ替代治疗患者的预后与非ＬＮ的ＥＳＲＤ替代治疗患者相同。当ＬＮ发展至不可逆的ＥＳＲＤ时,需长期作肾脏替代治疗,无必要再应用免疫抑制治疗。     

狼疮肾炎的病理与临床分析

目的探讨狼疮性肾炎（LN）的病理类型与临床及实验室检查的关系。方法对34例狼疮性肾炎患者进行肾活检及病理分型,分析各病理类型的临床特点、实验室检查特点、临床活动性及肾脏病理活动性。结果病理类型Ⅳ型最多见（占55．9％）,Ⅳ型LN肾功能不全及血尿发生率高,肾脏病理活动性明显;Ⅳ型、Ⅴ型、Ⅴ＋Ⅳ、Ⅴ＋ⅢLN肾病综合征、大量蛋白尿发生率高。肾脏病理活动指数与系统性红斑狼疮的临床活动性及抗dsDNA抗体阳性率无相关性。结论狼疮性肾炎的病理类型与临床表现、实验室检查有一定联系;但二者之间关系不确定。     

Roles of tumour necrosis factor‐related weak inducer of apoptosis/fibroblast growth factor‐inducible 14 pathway in lupus nephritis

As one of the manifestations of patients with systemic lupus erythematosus, lupus nephritis (LN) has high morbidity and mortality. Although the explicit mechanism of LN remains to be fully elucidated, there is increasing evidence to support the notion that tumour necrosis factor‐related weak inducer of apoptosis (TWEAK), acting via its sole receptor, fibroblast growth factor‐inducible 14 (Fn14), plays a pivotal role in such pathologic process. TWEAK/Fn14 interactions occur prominently in kidneys of LN, inducing inflammatory responses, angiogenesis, mesangial proliferation, filtration barrier injuries, renal fibrosis, etc. This review will specify the important roles of TWEAK/Fn14 pathway in the pathogenesis of LN with experimental data from cellular and animal models. Additionally, the raised levels of urinary and serum soluble TWEAK correlate with renal disease activity in patients with LN. The neutralizing antibodies targeting TWEAK or other approaches inhibiting TWEAK/Fn14 signals can attenuate renal damage in the murine lupus models. Therefore, to focus on TWEAK/Fn14 signalling may be promising in both clinical evaluation and the treatment of patients with LN.     

Renal haemodynamic characteristics and their correlation with renal pathology in patients with systemic lupus erythematosus

SUMMARY: To clarify the characteristics of renal haemodynamics and their correlation with renal pathology in patients with systemic lupus erythematosus (SLE), renal function and renal biopsy findings from 101 SLE patients were analysed retrospectively. Glomerular filtration rate (GFR) and renal plasma flow (RPF) were examined simultaneously. Filtration fraction (FF) was calculated from the values obtained for GFR and RPF (GFR/RPF). The GFR was low in one-third and within normal limits in two-thirds of class IV patients with lupus nephritis (LN). In contrast, high RPF was observed in half of class IV patients. As a result, over 70% of class IV patients possessed a very low FF (less than 15%). The sensitivity of very low FF for class IV LN was significantly higher than that of low GFR. In conclusion, low FF was frequently recognized, especially in patients with diffuse proliferative LN. Decreased FF was a highly sensitive indicator of diffuse proliferative LN. Thus, determination of renal haemodynamics, including FF, may be a useful clinical parameter for evaluating renal involvement in patients with SLE.     

Increased excretions of beta2-microglobulin, IL-6, and IL-8 and decreased excretion of Tamm-Horsfall glycoprotein in urine of patients with active lupus nephritis

Tubulointerstitial nephritis is a less frequently recognized but important complication of systemic lupus erythematosus. We have investigated the cytokine beta2-microglobulin (beta2M) and Tamm-Horsfall glycoprotein (THG) excretions in the urine of systemic lupus erythematosus patients to identify indices for evaluation of tubulointerstitial inflammation in lupus nephritis (LN). Daily urine was collected from 15 patients with active LN, from 12 patients with inactive LN, and from 17 normal subjects. The amounts of soluble interleukin (IL) 2 receptor, IL-6, IL-8, beta2M, and THG in urine were measured. Beta2M and THG were regarded as indicators of proximal and distal renal tubule function, respectively. The urinary excretions of IL-6 and IL-8 were significantly higher in patients with active LN than in those with inactive LN and in normal individuals. The excretion of soluble IL-2 receptor in all three groups of subjects was not significantly different. On the other hand, the excretion of beta2M in patients with LN was significantly higher than that in normal individuals. The excretion of beta2M in patients with active or inactive LN was not significantly different. The THG excretion was lower in patients with active LN and tubulointerstitial inflammation as compared with patients with inactive LN or normal individuals. Six patients underwent pulse cyclophosphamide therapy during the course of experiments. Five of them showed a decrease in IL-8 and IL-6 excretions in urine after the treatment. The excretions of beta2M and THG in urine, in addition to IL-6 and IL-8, can reflect the renal inflammatory activity in patients with lupus tubulointerstitial nephritis as well as in those having lupus glomerulonephritis.     

Outcome of lupus nephritis in Iranian children: prognostic significance of certain features

The objective of this study was to determine the clinical and histopathological features and outcome of children with lupus nephritis (LN). Of 84 children with systemic lupus erythematosus (SLE), we retrospectively studied 58 children (69%) under 15 years of age with biopsy-proven LN who had been followed between October 1989 and January 2005. The mean age at diagnosis or initial referral was 10.6 ± 2.25 years, and the mean follow-up was 5.3 ± 4.1 years. Class IV LN was observed in 34 (58.6%) patients. The 5-year patient and renal survival rates were 82.5 and 78.5%, respectively, in the total group, and 75 and 85.8%, respectively, in patients with Class IV LN. No independent predictor of unfavorable outcome, including renal histology, was detected by multivariate analysis. The mid-term patient and the renal survival rates of Iranian children with biopsy-proven LN are high. Within 5 years of follow-up, renal histology was not a predictor for survival.