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IgA系膜增生性肾小球肾炎;节段性-局灶性肾小球病变的意义及发病机制

IGA mesangial glomerulonephritis; significance and pathogenesis of segmental-focal glomerular lesions.

作者信息

Syré G

出版信息

Virchows Arch A Pathol Anat Histopathol. 1983;402(1):11-24. doi: 10.1007/BF00695045.

DOI:10.1007/BF00695045
PMID:6419453
Abstract

A review of 430 renal biopsies from patients with various nephropathies processed by light microscopy, immunofluorescence and in part by electron microscopy revealed 82 cases with diffuse mesangial IgA deposition. Sixty-three cases appearing without signs of systemic disease were included in this study. The glomerular changes consisted of mesangial increase (31 cases mild, and 32 cases severe) and segmental-focal glomerular lesions (40 cases). Immunofluorescence revealed granular deposits of immunoglobulins and complement within the mesangium (IgG 9, IgM 25, IgA 63, C3 27, C1q 0) as well as segmentally in the wall of capillary loops (IgG 4/26, IgM 20/26, IgA 0/26, C3 16/26, C1q 7/26). Electron microscopic studies (40 cases) showed electron dense deposits in the mesangial region in all biopsies and deposits either electron dense (11 cases) or radiolucent (26 cases) at various sites along the basement membrane of capillary loops. Comparison between morphological findings and clinical data showed a significant correlation between segmental-focal glomerular lesion on the one hand, and proteinuria (p less than 0.01), immune deposits along the capillary basement membrane (p less than 0.01), and the increased mesangium (p less than 0.001) on the other. Considering the macromolecular mesangial clearing function, the segmental-focal glomerular lesions may be due to newly arrived, infection related immune complexes which may not be cleared promptly by the mesangium, because its clearing function is impaired due to IgA deposition.

摘要

对430例患有各种肾病的患者的肾活检组织进行光学显微镜、免疫荧光检查,部分进行电子显微镜检查,结果显示82例存在弥漫性系膜IgA沉积。本研究纳入了63例无全身疾病体征的病例。肾小球病变包括系膜增生(轻度31例,重度32例)和节段性-局灶性肾小球病变(40例)。免疫荧光显示免疫球蛋白和补体在系膜内呈颗粒状沉积(IgG 9例,IgM 25例,IgA 63例,C3 27例,C1q 0例),以及在毛细血管袢壁节段性沉积(IgG 4/26,IgM 20/26,IgA 0/26,C3 16/26,C1q 7/26)。电子显微镜研究(40例)显示,所有活检组织的系膜区均有电子致密沉积物,在毛细血管袢基底膜的不同部位有电子致密沉积物(11例)或透亮沉积物(26例)。形态学发现与临床数据的比较显示,一方面节段性-局灶性肾小球病变与蛋白尿(p<0.01)、毛细血管基底膜免疫沉积物(p<0.01)以及系膜增生(p<0.001)之间存在显著相关性。考虑到系膜的大分子清除功能,节段性-局灶性肾小球病变可能是由于新到达的、与感染相关的免疫复合物,系膜可能无法及时清除这些复合物,因为其清除功能因IgA沉积而受损。

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引用本文的文献

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An acute model for IgA-mediated glomerular inflammation in rats induced by monoclonal polymeric rat IgA antibodies.一种由单克隆聚合大鼠IgA抗体诱导的大鼠IgA介导的肾小球炎症急性模型。
Clin Exp Immunol. 1993 Jun;92(3):514-21. doi: 10.1111/j.1365-2249.1993.tb03430.x.
2
Mesangial cell autoantigens in immunoglobulin A nephropathy and Henoch-Schönlein purpura.免疫球蛋白A肾病和过敏性紫癜中的系膜细胞自身抗原。
J Clin Invest. 1991 Nov;88(5):1522-30. doi: 10.1172/JCI115462.

本文引用的文献

1
Immunologic studies in IgA nephropathy.IgA肾病的免疫学研究。
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Renal disease in Singapore with particular reference to glomerulonephritis in adults.新加坡的肾脏疾病,特别提及成人肾小球肾炎
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Impaired mesangial clearance of macromolecules in rats with chronic mesangial ferritin-antiferritin immune complex deposition.慢性肾小球系膜铁蛋白 - 抗铁蛋白免疫复合物沉积大鼠中大分子物质的肾小球系膜清除受损。
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Idiopathic mesangial IgA-glomerulonephritis in childhood Description of 19 pediatric cases and review of the literature.儿童特发性系膜IgA肾小球肾炎:19例儿科病例描述及文献综述
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Is there a treatment of mesangial IgA glomerulonephritis?
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IgA-IgG nephropathy: predictive indices of progressive disease.IgA-IgG肾病:疾病进展的预测指标
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IgA nephropathy.IgA肾病
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Schweiz Med Wochenschr. 1973 May 26;103(21):761-8.
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IgA glomerular deposits in renal disease.肾病中的IgA肾小球沉积物。
Transplant Proc. 1969 Dec;1(4):939-44.