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IgA肾病和过敏性紫癜患者血清IgA-纤连蛋白聚集体:诊断价值及致病意义。肾小球疾病协作网络。

Serum IgA-fibronectin aggregates in patients with IgA nephropathy and Henoch-Schönlein purpura: diagnostic value and pathogenic implications. The Glomerular Disease Collaborative Network.

作者信息

Jennette J C, Wieslander J, Tuttle R, Falk R J

机构信息

Department of Pathology, School of Medicine, University of North Carolina, Chapel Hill 27599.

出版信息

Am J Kidney Dis. 1991 Oct;18(4):466-71. doi: 10.1016/s0272-6386(12)80115-6.

Abstract

IgA nephropathy is a common form of glomerulonephritis that has varied clinical expressions, ranging from asymptomatic hematuria to rapidly progressive nephritis. We report the strong association (P less than 0.0001) of circulating IgA-fibronectin aggregates with IgA nephropathy. Of 30 patients with IgA nephropathy, 93.3% had serum IgA-fibronectin aggregates detected with an enzyme immunoassay using collagen as a substrate to bind the aggregates. Among the patients with IgA-fibronectin aggregates were patients with Henoch-Schönlein purpura and recurrent crescentic IgA nephropathy in transplants. Only 11.7% of 103 patients with other types of glomerular disease and 6.7% of normal controls had positive IgA-fibronectin aggregate assay levels. IgA-fibronectin aggregates also were detected in serum using an antifibronectin antibody capture assay; and could be depleted from serum by heparin-agarose affinity chromatography. The circulating IgA-fibronectin aggregates had the same unusual predominance of lambda relative to kappa light chains that is observed in the glomerular deposits of IgA nephropathy. The data indicate that IgA-fibronectin aggregates are a useful serologic marker for IgA nephropathy, Henoch-Schönlein purpura, and recurrent IgA nephropathy in transplants. The presence of fibronectin in the circulating aggregates may play an important role in the preferential deposition of nephritogenic IgA-containing immune complexes in the mesangium of patients with IgA nephropathy.

摘要

IgA肾病是一种常见的肾小球肾炎形式,临床表现多样,从无症状血尿到快速进展性肾炎不等。我们报告循环IgA-纤连蛋白聚集体与IgA肾病有很强的相关性(P小于0.0001)。在30例IgA肾病患者中,93.3%通过以胶原蛋白为底物结合聚集体的酶免疫测定法检测到血清IgA-纤连蛋白聚集体。在有IgA-纤连蛋白聚集体的患者中,包括过敏性紫癜患者和移植后复发性新月体性IgA肾病患者。103例其他类型肾小球疾病患者中只有11.7%以及正常对照者中有6.7%的IgA-纤连蛋白聚集体检测水平呈阳性。还使用抗纤连蛋白抗体捕获测定法在血清中检测到IgA-纤连蛋白聚集体;并且可以通过肝素-琼脂糖亲和层析从血清中去除。循环中的IgA-纤连蛋白聚集体具有与IgA肾病肾小球沉积物中观察到的相同的λ相对于κ轻链的异常优势。数据表明,IgA-纤连蛋白聚集体是IgA肾病、过敏性紫癜和移植后复发性IgA肾病的一种有用的血清学标志物。循环聚集体中纤连蛋白的存在可能在IgA肾病患者系膜中致肾炎性含IgA免疫复合物的优先沉积中起重要作用。

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