纤维样肾小球病病理分析

Routine pathological analysis of fibrillary glomerulopathy

目的:光镜及电镜下观察纤维样肾小球病的病理特征,为纤维样肾小球病的诊断及鉴别诊断提供依据。方法:2例纤维样肾小球病患者(其中1例并发免疫触须样肾小球病)经皮肾穿刺取活检组织,HE、PAS、PAM-Masson染色,IgG、IgM、IgA、C3、C4、C1q免疫组化染色,于光镜下观察;病理分析(光镜和电镜)及免疫组织化学染色;铀铅双重染色透射电镜观察。结果:光镜下PAM-Masson染色膜性肾病示系膜区团块状、肾小球基底膜细小嗜复红蛋白沉积,而膜增殖性肾小球肾炎示肾小球基底膜局灶增厚、"双轨化"伴嗜复红蛋白沉积。免疫组织化学染色膜性肾病和膜增殖性肾小球肾炎在肾小球系膜区有IgG、IgM、IgA、C3免疫复合物沉积,而在血管襻区膜性肾病见IgG、C3免疫复合物沉积,膜增殖性肾小球肾炎可见IgM、C3、C1q免疫复合物沉积,但其刚果红染色阴性。电镜下膜性肾病和膜增殖性肾小球肾炎在系膜区、基底膜上皮下和(或)内皮下有电子致密物沉积,见无分支的纤维样结构(直径15～25nm),而膜增殖性肾小球肾炎还可见微管状结构(直径30～50nm)。结论:纤维样肾小球病具有典型的形态学特征,确诊需依赖电镜特征性纤维样结构。

Objective To study the pathologic and ultrastructural features of fibrillary glomerulopathy（FGP）,and provide basis for diagnosis and differential diagnosis of FGP.Methods Two renopuncture biopsy tissues of FGP （one case combined immunotactoid glomerulopathy）were studied by routine histopathological（morphologically and ultrastructurally）and were observed by light microscope with staining （HE,PAS,PAM-Masson）and immunohistochemical staining （IgG,IgM,IgA,C3,C4,C1q）and by electron microscope with Lead citrate-Uranyl acetate staining.Results Morphologically,membranous nephropathy with immune reactants were showed in mesangium and subepithelial areas of glomerular basement membrane （GBM）in membranous glomerulopathy （MG）and membranoproliferative glomerulonephritis （MPGN）with mesangial nodular sclerosis and GBM＇s double-track,which immune reactants deposited showed in subendothelial areas of GBM in MPGN by PAM-Masson staining.Renal mesangium of MG and MPGN associated with deposition of material that was positive for IgG,IgM,IgA,C3,capillary tuft of MG was positive for IgG,C3and capillary tuft of MPGN was positive for IgM,C3,C1q by immunohistochemical staining,but they were not stained by Congo red.Ultrastructurally,the mesangial,subepithelial and/or subendothelial areas of MG and MPGN were expanded because of the electron-dense deposits,which were represented by randomly oriented nonbranching fibrils with a diameter of about 15-25nm.In addition,microtubule deposits with a diameter of about 30-50nm were also found in MPGN.Conclusion The diagnosis of FGP is always based on biopsy of the kidney which revealed the characteristic of fibrils composed of immune reactants under electron microscope.