Watanabe Kentaro, Nakai Kentaro, Hosokawa Nozomi, Watanabe Shuhei, Kono Keiji, Goto Shunsuke, Fujii Hideki, Hara Shigeo, Nishi Shinichi
Division of Nephrology and Kidney Center, Kobe University Graduate School of Medicine, Kobe, Japan.
Department of Nephrology and Kidney Center, Kakogawa Central City Hospital, Kakogawa, Japan.
Case Rep Nephrol Dial. 2017 Apr 5(1):26-33. doi: 10.1159/000468517. eCollection 2017 Jan-Apr.
Herein, we report a case of fibrillary glomerulonephritis (FGN). FGN usually shows non-amyloidal fibrils in the mesangium and glomerular capillary walls on electron microscopy. Inherited cases of FGN have been reported in only 3 families, and the suspected genetic form was autosomal dominant. In the present case, the deposition of microfibrils in the arteriolar wall as well as the glomerulus is unique. Our patient's father died of nephrotic syndrome, and his elder brother had a biopsy-proven glomerulopathy. The histological findings of the brothers are similar to mesangial proliferative glomerulonephritis and resemble each other. Therefore, our case is presumed to be familial FGN. Additionally, herein, we review the literature and reconsider the histological and clinical characters of FGN.
在此，我们报告一例纤维性肾小球肾炎（FGN）病例。FGN在电子显微镜下通常显示系膜和肾小球毛细血管壁中有非淀粉样纤维。仅在3个家族中报道过FGN的遗传病例，推测的遗传形式为常染色体显性遗传。在本病例中，微纤维在小动脉壁以及肾小球中的沉积是独特的。我们患者的父亲死于肾病综合征，他的哥哥经活检证实患有肾小球病。兄弟俩的组织学表现类似于系膜增生性肾小球肾炎，且彼此相似。因此，我们的病例被推测为家族性FGN。此外，在此我们回顾文献并重新审视FGN的组织学和临床特征。
