免疫触须样肾小球病的病理形态观察及鉴别诊断

Pathology and differential diagnosis of immunotactoid glomerulopathy

目的了解免疫触须样肾小球病的形态学改变，并对其临床病理特征及鉴别诊断进行探讨。方法对确诊的l例免疫触须样肾小球病肾活检组织进行光镜、免疫荧光、电镜观察。结果患者临床表现为肾病综合征伴高血压，无肾病家族史。光镜检查：Masson染色示肾小球弥漫性团块状特殊蛋白沉积。免疫荧光：IgG、C3、K沿毛细血管壁沉积和系膜区团块状沉积。电镜检查在毛细血管壁内皮细胞下和系膜区见大量微管状物质和少量长杆状致密结晶样物质沉积。结论免疫触须样肾小球病的光镜和免疫荧光无明显特殊病理改变，电镜下肾小球内大量微管状物质沉积，是本病区别于其他肾小球疾病的关键之点。该例患者除大量微管状物质沉积外，同时伴有长杆状致密结晶样物质沉积。

Objective To study the morphologic changes of immunotactoid glomerulopathy and to investigate the clinical pathological features and differential diagnosis. Methods Renal biopsy was observed under the light microscope, immunofluorescence and electron microscopy in a case of newly diagnosed immunotactoid glomerulopathy. Results This patient clinically presented with nephrotic syndrome and hypertension, without family history of renal diseases. Light microscopy showed that diffusely massive and specific protein deposition in the glomerulus in Masson staining. Immundluorescence revealed IgG, and K were deposited along the capillary walls and mesangial regions. Electron microscopic examination showed that a large amount of micretubule like substances and a small amount of long bar-shaped and dense crystal-like substances were deposited in the subendothelial spaces and mesangial areas. Condusions Light microscopy and immunofluorescence of immunotactoid glomerulopathy show no specifically pathological changes. Under electron microscope, a large amount of microtubule like substances is deposited in the glomerulus, which is the key point to distinguish this disease from other glomerular diseases. Except for the microtubule-like substances, the present case is accompanied by the deposition of long bar-shaped and dense crystal-like substance, which has not been reported in previous studies.