Watanabe Sayaka, Arimura Yoshihiro, Nomura Kazufumi, Kawashima Soko, Yoshihara Ken, Kaname Shinya, Yamada Akira
First Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan.
Nihon Jinzo Gakkai Shi. 2011;53(1):46-52.
We experienced a rare case of membranous glomerulopathy(MN) with myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-associated glomerulonephritis. A 79-year-old woman was admitted to our hospital because of pyrexia, microscopic hematuria, massive proteinuria and positive MPO-ANCA on June, 2007. We diagnosed her as MPO-ANCA-associated vasculitis accompanied by nephrotic syndrome. Intravenous methylprednisolone sodium succinate (500 mg/day for three days)therapy and oral prednisolone (40 mg/day) improved her fever, hematuria, serum CRP and MPO-ANCA titer. Renal biopsy was performed and light microscopic examination of a renal biopsy specimen containing 21 glomeruli revealed global sclerosis in 3 and thickened basement membrane in 18 of the glomeruli. Fibrocellular crescents were found in 2 and segmental necrosis in 1. Immunofluorescence microscopy showed granular staining with IgG and C3 along the capillary walls. Electron microscopic examination disclosed subepithelial dense deposits in the thickened glomerular basement membrane. To investigate the pathogenesis of MN, IgG subclass was examined by means of immunofluorescence microscopy. IgG1 and IgG4 were deposited on the glomerular capillary walls, which suggested secondary MN. However, this patient refused to take any medicines and had no disease such as infection or cancer which cause secondary MN. MPO staining was performed to investigate the relation of MPO-anti-MPO antibody immune complex in the pathogenesis of MN. The results showed only a few MPO-positive cells in the glomeruli and MPO stains on the glomerular capillary walls near the MPO-positive cells. These findings suggested that the patient had MPO-ANCA-associated glomerulonephritis superimposed on idiopathic MN. In the case of nephrotic syndrome with MPO-ANCA, we should consider the coexistence of other types of glomerulonephritis, especially MN.
我们遇到了一例罕见的膜性肾小球病(MN)合并髓过氧化物酶抗中性粒细胞胞浆抗体(MPO-ANCA)相关肾小球肾炎的病例。一名79岁女性于2007年6月因发热、镜下血尿、大量蛋白尿及MPO-ANCA阳性入住我院。我们诊断她为MPO-ANCA相关血管炎伴肾病综合征。静脉注射甲泼尼龙琥珀酸钠(500mg/天,共三天)及口服泼尼松龙(40mg/天)治疗改善了她的发热、血尿、血清CRP及MPO-ANCA滴度。进行了肾活检,对含有21个肾小球的肾活检标本进行光镜检查,发现3个肾小球全球硬化,18个肾小球基底膜增厚。2个肾小球有纤维细胞性新月体形成,1个有节段性坏死。免疫荧光显微镜检查显示沿毛细血管壁有IgG和C3颗粒状染色。电镜检查发现增厚的肾小球基底膜有上皮下致密沉积物。为研究MN的发病机制,通过免疫荧光显微镜检查了IgG亚类。IgG1和IgG4沉积在肾小球毛细血管壁上,提示为继发性MN。然而,该患者拒绝服用任何药物,也没有导致继发性MN的感染或癌症等疾病。进行MPO染色以研究MPO-抗MPO抗体免疫复合物在MN发病机制中的关系。结果显示肾小球内仅有少数MPO阳性细胞,且MPO阳性细胞附近的肾小球毛细血管壁有MPO染色。这些发现提示该患者患有MPO-ANCA相关肾小球肾炎叠加在特发性MN之上。对于伴有MPO-ANCA的肾病综合征病例,我们应考虑其他类型肾小球肾炎的共存,尤其是MN。