纤维样肾小球肾炎的临床病理特征
任雅丽，徐 进，张玉洁，张 旭，黄晨诗，程 明，周 晶，王素霞*纤维样肾小球肾炎的临床病理特征
任雅丽，徐 进，张玉洁，张 旭，黄晨诗，程 明，周 晶，王素霞*
（北京大学第一医院电镜室，北京 100034）
摘 要 目的：了解罕见病纤维样肾小球肾炎的临床病理特征。方法：选取我室经常规透射电镜确诊为纤维样肾小球肾炎的10例患者，对其临床病理特征进行回顾性总结和分析。结果：电镜下纤维结构的沉积部位以肾小球基底膜致密层内最常见（100%），其次为系膜区（90%）和内皮下（30%），未见上皮下沉积，所有的纤维性沉积物仅限于肾小球内。低倍镜下病变区域呈现与普通免疫性电子致密物相似的沉积，密度低等至中等，有模糊云雾状表现；高倍下见直径为15~30 nm的纤维结构杂乱无序地稀疏分布。90%患者上皮足突融合达80%~100%。光镜下最常见的病理类型为系膜增生性肾小球肾炎。免疫荧光染色显示肾小球内多克隆性免疫球蛋白沉积，常伴有补体沉积。行DNAJB9免疫电镜标记的所有病例均呈阳性表达。结论：纤维样肾小球肾炎最常累及的部位是肾小球基底膜的致密层，免疫电镜是可靠的辅助诊断方法。
关键词 纤维样肾小球肾炎；透射电镜；纤维；免疫电镜
中图分类号：R365；R692.3；Q336
文献标识码：A Doi:10.3969/j.issn.1000-6281.2025.06.006
The clincopathological features of fibrillary glomerulonephritis
REN Yali，XU Jin，ZHANG Yujie，ZHANG Xu，HUANG Chenshi，CHENG Ming，ZHOU Jing，WANG Suxia*
(Laboratory of Electron Microscopy, Peking University First Hospital, Beijing 100034, China)
Abstract Objective：To investigate the clinicopathological characteristics of renal biopsy in patients with fibrillary glomerulonephritis (FGN). Method: Ten patients diagnosed with FGN by transmission electron microscopy in Laboratory of Electron Microscopy of Peking University First Hospital were selected, and their clinical and pathological characteristics were retrospectively summarized and analyzed. Result: By electron microscopy, fibrillary deposits of disorderly distributed 15 to 30 nm diameter were found in lamina densa of the glomerular basement membrane (GBM) in all patients and in mesangium of 90% cases. Three cases had significant subendothelial deposition. Subepithelial deposition was absent. No deposition outside the glomeruli was observed. Low to medium density materials with mottled appearance was noted under low magnification. By higher magnification the fiber is more pronounced in areas with medium electron density. 90% cases showed diffuse foot process effacement ranging from 80% to 100%. Under light microscopy, mesangial proliferative glomerulonephritis is the most common pathological type. Immunofluorescence staining showed polyclonal immunoglobulin deposition, often accompanied by complement deposition. All samples labeled with DNAJB9 immunoelectron microscopy showed positive results. Conclusion: The most commonly affected location of FGN is the lamina densa of the GBM, and immunoelectron microscopy is a reliable diagnostic method.
Keywords fibrillary glomerulonephritis; transmission electron microscopy; fiber; immunoelectron microscopy