胶原Ⅲ肾病——形态学特点和临床表现  

Collagen type Ⅲ glomerulopathy:Morphological features and clinical manifestations

作　　者：陈惠萍[1] 徐峰[1] 黄倩[1] 黄高渊[1] 谢红浪[1] 朱茂艳[1] 张明超[1] 贺倩[1] 梁少姗[1] 曾彩虹[1] 刘志红[1] 

机构地区：[1]南京军区南京总医院全军肾脏病研究所,南京210002

出　　处：《肾脏病与透析肾移植杂志》2011年第6期522-529,共8页Chinese Journal of Nephrology,Dialysis & Transplantation

摘　　要：目的:总结一种非免疫因素介导的肾小球疾病——胶原Ⅲ肾病的肾活检形态学及实验室检查特点,旨在提高对此病的诊断水平。方法:回顾性分析经肾活检免疫荧光、电镜检查确诊胶原Ⅲ肾病的12例患者的组织学特点,并分析其临床表现及实验室检查结果。结果:12例患者男性4例,女性8例,病程3～300月(平均62.08±60.96月);年龄21～67岁。蛋白尿为本组患者最常见的肾脏损害首发症状(10例,83.33%),10例(83.33%)患者病初即诊断高血压,尿沉渣红细胞计数>50万/ml者仅1例(8.33%)。肾小管受损的实验室指标包括尿NAG酶升高9例(75%);禁水13h尿渗量减低者9例。确诊时血清肌酐(SCr)升高者4例,7例贫血。肾活检免疫荧光检查C3阳性者5例(41.67%),3例(25%)患者合并IgA阳性,胶原Ⅲ染色证实非废弃或非硬化部位的肾小球外周袢和(或)系膜区弥漫阳性荧光;组织学观察证实,12例患者肾小球体积均增大,且肾小球内皮下疏松、区域增宽等,肾小球病变大致分为两种类型,一类肾小球非增生性的"结节样"改变,PAS或PASM-Masson染色肾小球毛细血管袢内皮下呈均质、淡染的嗜复红或嗜亮绿色;另种"结节样"改变不明显,肾小球细胞总数不多或增加,内皮细胞成对,PASM-Masson染色外周袢弥漫双轨。超微结构观察证实12例患者肾小球毛细血管袢内皮下疏松、区域增宽,内皮下和(或)系膜区见束状或单枝的、有明暗带、直径约43～60nm的胶原纤维,3例(25%)免疫荧光染色IgA阳性者在肾小球系膜区见团块状的电子致密物沉积。随访过程中病初4例(33.3%)SCr升高者未降至正常,病初SCr正常者中仅1例在随访中轻度增高。结论:高血压和蛋白尿是胶原Ⅲ肾病最常见的临床表现,肾活检组织学改变具特征性,诊断需借助免疫荧光染色和超微结构观察;预后相对良好。Objective：Collagen type m glomerulopathy is a non-immune-mediated glomerular disease, and also a form of glomerulopathy in which organized collagen type Ⅲ progressively deposits. We analyzed the renal morphological features and laboratory examinations,as well as clinical manifestations, in patients with collagen type m glomerulopathy to improve diagnostic level of this disease. Methodology ：Twelve cases with collagen type Ⅲ glomerulopathy proved by light microscopy,immunofluorescence and electron microscopy were retrospectively studied. Their clinical materials, laboratory findings and pathological data were collected. Results：There were 4 males and 8 females aged from 21 to 67 years old. The mean duration of disease was （62. 1 ± 60. 9） months （ ranged from 3 to 300 months）. Proteinuria and hypertension were the most common onset clinical symptoms with the incidence both of 83.3%. Most cases had normal renal function at making diagnosis except four cases （33.3%）. The increase of red blood cell in urinary sediment was presented in only one case （8.3%）. Most cases had tubular injury including elevated urine NAG in 9 （75.0%）, increased urine RBP in 4 and decreased urine osmolality in all cases. Immunofluorescence microscopy revealed that collagen type Ⅲ was abundant staining in the non-sclerosis glomerular capillary loops and/or mesangium, and C3 and IgA were positive in 5 （41.7%） and 3 （25.0%） cases,respectively. In the light microscopy,glomerular tufts were globally expanded and the subendothelial spaces of glomerutar basement membrane were markedly expanded in all cases. There were two typic patterns in glomerular changes. One was that the globular deposition of eosinophilic substances in the expanded mesangial area was observed,narrowing of capillary lumen in glomerulus and a decrease in nuclearity of the mesangial area and endothelium was noted, and Bowman＇s capsules were thickened. Another was the marked enlargement of the glomerular tuft due to both expansion of the

关 键 词：胶原Ⅲ肾病 肾活检 高血压 蛋白尿 

分 类 号：R692[医药卫生—泌尿科学]