ISSN 1006-298X      CN 32-1425/R

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肾脏病与透析肾移植杂志 ›› 2016, Vol. 25 ›› Issue (5): 489-493.DOI: 10.3969/cndt.j.issn.1006-298X.2016.05.019

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IgA型免疫管状肾小球病

  

  • 出版日期:2016-10-28 发布日期:2016-11-03

Immunotactoid glomerulopathy with prevalent IgA deposition

  • Online:2016-10-28 Published:2016-11-03

摘要:

老年男性患者,病程6年,肾脏损伤表现为肾病综合征、大量镜下血尿,肾功能不全及高血压,肾外表现为反复发作的双下肢紫癜样皮疹、补体C3偏低、贫血、血轻链κ/λ比值增高。肾活检组织学改变为肾小球膜增生样病变,免疫荧光以IgA沿肾小球毛细血管袢沉积为主,电镜下肾小球系膜区、内皮下、上皮侧见平行排列、直径均一的中空微管状物质。该患者最终诊断为IgA型免疫管状肾小球病。

Abstract:

A 65yearold man who had recurrent skin purpura rash developed nephrotic symdrome, hematuria, renal failure, hypertension, as well as extrarenal manifestations of anemia, decreased C3, and elevated κ∶λ ratio. Renal biopsy showed membranoproliferative glomerulonephritis (MPGN). Immunoflurerscence indicated granular staining of capillary loop and mesangium in glomeruli for IgA, κ and λ light chain. Ultrastructral examination showed presence of electrondense deposition of microtubules that had distinct hollow centers with 3347 nm in diameter, arranged in parallel in the mesangium, subendothelial and rarely in the subepithelial area. The final diagnosis was immunotactoid glomerulopathy with prevalent IgA deposition.