伴有补体缺陷的IgA肾病

IgA nephropathy with complement deficiency.

作者信息

Kanda E, Shimamura H, Tamura H, Uchida S, Terada Y, Sakamoto H, Kuwabara M, Akiba T, Ida T, Sasaki S, Marumo F

机构信息

Department of Internal Medicine, Tokyo Medical and Dental University School of Medicine.

出版信息

Intern Med. 2001 Jan;40(1):52-5. doi: 10.2169/internalmedicine.40.52.

DOI:10.2169/internalmedicine.40.52

PMID:11201372

Abstract

We treated a female patient suffering from immunoglobulin A (IgA) nephropathy and congenital deficiency of the ninth component of the complement system (C9). She was admitted with hematuria and proteinuria, and the C9 deficiency was diagnosed based on the low hemolytic activity of 50 % of the hemolytic unit of the complements (CH50) and the normal C3 level in the plasma. Renal biopsy revealed mild mesangial proliferation, and immunofluorescence examination revealed mild mesangial deposits of IgA and C3 with the same distribution. We discuss the pathogenesis of IgA nephropathy and the role of the complements in its progression.

摘要

我们治疗了一名患有免疫球蛋白A（IgA）肾病和补体系统第九成分（C9）先天性缺乏症的女性患者。她因血尿和蛋白尿入院，根据补体50%溶血活性（CH50）低及血浆中C3水平正常诊断为C9缺乏症。肾活检显示轻度系膜增生，免疫荧光检查显示IgA和C3在系膜轻度沉积且分布相同。我们讨论了IgA肾病的发病机制以及补体在其进展中的作用。