抗中性粒细胞胞浆抗体相关性血管炎,淀粉样变性," /> 抗中性粒细胞胞浆抗体相关性血管炎,淀粉样变性,"/> <b>ANCA</b><b>相关血管炎继发肾淀粉样变性</b><b>:<span>1</span>例病例报告</b>

Chinese Medical Sciences Journaldoi: 10.24920/003999

• 病例报告 •    下一篇

ANCA相关血管炎继发肾淀粉样变性1例病例报告

何鑫1,宁建平2,许辉2,肖宫2,阳惠湘1,王维圆3,吴晓英3,尹红玲3,李晓照2*   

  1. 1中南大学湘雅医院消化内科,2肾内科,3病理科,长沙 410008

Renal amyloidosis secondary to ANCA-associated vasculitis: a case report

Xin He1, Jianping Ning2, Hui Xu2, Gong Xiao2, Huixiang Yang1, Weiyuan Wang3, Xiaoying Wu3, Hongling Yin3, Xiaozhao Li2*   

  1. 1Department of Gastroenterology, 2Department of Nephrology, 3Department of Pathology, Xiangya Hospital, Central South University, Changsha 410008, China

摘要: 摘要:临床上继发于抗中性粒细胞胞浆抗体anti-neutrophil cytoplasmic antibodyANCA相关血管炎的淀粉样蛋白Aamyloid AAA)型淀粉样变性病例十分罕见。我们收治了177岁的ANCA相关血管炎女性患者。经活检取肾组织标本,Masson三色染色提示寡免疫复合物型新月体性肾小球炎;在电子显微镜下观察可见肾小球系膜区淀粉样物质沉积;免疫荧光染色显示λ轻链和κ轻链染色阴性。骨髓活组织检查未见浆细胞异常增生。最终,我们做出ANCA相关血管炎多血管炎继发AA型淀粉样变的诊断。

关键词: 抗中性粒细胞胞浆抗体相关性血管炎')">抗中性粒细胞胞浆抗体相关性血管炎, 淀粉样变性

Abstract: Abstract: Renal amyloidosis secondary to anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is extremely rare. Here, we reported a 77-year-old woman with ANCA-associated vasculitis. Renal biopsy with Masson trichrome staining showed pauci-immune crescentic glomerulonephritis, and electron microscopy showed amyloid deposition in the mesangial area. Immunofluorescence revealed kappa light chain and lambda light chain were negative. Bone marrow biopsy revealed no clonal plasma cell. Finally, she was diagnosed as ANCA-associated vasculitis with secondary renal amyloid A amyloidosis.

Key words: anti-neutrophil cytoplasmic antibody-associated vasculitis, amyloidosis

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