触须样免疫性肾小球病的临床病理观察

Immunotactoid glomerulopathy

目的 了解国内触须样免疫性肾小球病（ｉｍｍｕｎｏｔａｃｔｏｉｄ ｇｌｏｍｅｒｕｌｏｐａｔｈｙ， ＩＴ）的发病及其临床病理特点。方法应用电子显微镜观察了北京医科大学第一医院肾内科１９９２－１９９５年所做肾活检６００例患者,其中３例符合ＩＴ，并通过临床分析，光学显微镜，免疫荧光检查，刚果红染色及图像分析技术等，除外了淀粉样变性病，纤维样肾小球病及其他系统性疾病。结果３例患者ＩＴ的超微病理特点：在肾小球不同部位及电子致密物中可见大量微管状纤维（直径３５－４７ｎｍ），并伴有直径约２０ｎｍ的紊乱排列的纤维样物质沉积，刚果红染色阴性，患者为中青年男性，有蛋白尿，镜下血尿及高血压，２例出现肾功能不全。结论（１）ＩＴ为少见肾病，我国也有该病发生；（２）与国外病例相比，该３例发病年龄较轻，预后较差；（３）病变中微管状和纤维样物质同时存在，说明ＩＴ和纤维样肾小球病可能为同一疾病的两种类型。

Objective To describe 3 patients with immunotactoid glomerulopathy ( IT ) and their clirtico- pathological features. Methods Renal biopsies were examined by EM ; they were collected during the last 3 years in Department of Nephrology , First Hospital of Beijing Medical University. The amyloido- sis , fibrillary glomerulopathy (FGP ) and other systemic disease were excepted by clinical analysis , LM , IF , Congo red staining and image pattern analysis. Results The ultrastructural characteristic of the three cases were microtubular fibrils (diameter : 35-47nm) deposited in various sites of glomeruli (GBM , subendothelium and mesangium ) and electron dense deposits. In addition , randomly fibrillary deposits ( diameter , 20nm ) were also found . The fibrils were negative with Congo red staining. The 3 patients were young middle-aged men with proteinuria , microscopic hematuria and hypertension , two of them had renal insufficiency. Conclusions IT is a rare nephropathy but exists in China. Our , patients , were younger Than reported and there prognosis were poor. The microtubular and fibrillary deposits appear in some cases , indicating that IT and FGP are different subtypes of an entity.