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IgA肾病的免疫组织学

The immunohistology of IgA nephropathy.

作者信息

Jennette J C

机构信息

Department of Pathology, School of Medicine, University of North Carolina, Chapel Hill 27599-7525.

出版信息

Am J Kidney Dis. 1988 Nov;12(5):348-52. doi: 10.1016/s0272-6386(88)80022-2.

Abstract

The glomerular immunohistologic characteristics of 180 patients with IgA nephropathy (IgAN), defined by 2+ or greater (out of 0 to 4+) mesangial IgA-dominant or codominant immunostaining and no evidence for systemic lupus erythematosus, were compared with those of 84 patients with proliferative lupus glomerulonephritis and 254 patients with other forms of proliferative glomerulonephritis. The IgAN population increased in number by only 5% if the IgA immunostaining criterion was lowered to 1+, and it decreased by only 2% if IgA codominant staining was disallowed. A distinctive immunohistologic feature of IgAN in comparison with other immune complex-mediated glomerulopathies, in addition to the predominance of IgA immunostaining, was a high frequency (67%) of patients with greater lambda- than kappa-immunoglobulin light chain immunostaining. There was no correlation between the absolute or relative intensities or frequencies of IgA, IgG, or IgM immunostaining and the severity of glomerular disease; however, the presence of capillary wall immune deposits correlated with more severe disease. Terminal complement components were consistently present and were more conspicuous in more severely injured glomeruli. Immunostaining for the early classical complement activation pathway component C1q was absent or scanty in IgAN. This finding was particularly useful in the immunohistologic differentiation of IgAN from proliferative lupus glomerulonephritis, which was the form of glomerulonephritis with the greatest overlap with IgAN with respect to IgA immunostaining. When the diagnostic criteria for IgAN were 2+ or greater, dominant or codominant mesangial IgA immunostaining and less than 2+ C1q immunostaining, an immunohistologic diagnosis of IgAN was made with 98% accuracy.

摘要

对180例IgA肾病(IgAN)患者的肾小球免疫组织学特征进行了研究,这些患者的定义为系膜区IgA为主或共显性免疫染色为2+或更高(0至4+),且无系统性红斑狼疮证据,并与84例增殖性狼疮性肾小球肾炎患者和254例其他形式的增殖性肾小球肾炎患者进行了比较。如果将IgA免疫染色标准降至1+,IgAN患者数量仅增加5%;如果不允许IgA共显性染色,患者数量仅减少2%。与其他免疫复合物介导的肾小球病相比,IgAN的一个独特免疫组织学特征,除了IgA免疫染色占优势外,是λ链免疫球蛋白轻链染色大于κ链的患者频率较高(67%)。IgA、IgG或IgM免疫染色的绝对或相对强度或频率与肾小球疾病的严重程度之间没有相关性;然而,毛细血管壁免疫沉积物的存在与更严重的疾病相关。终末补体成分始终存在,在损伤更严重的肾小球中更明显。IgAN中早期经典补体激活途径成分C1q的免疫染色缺失或稀少。这一发现对于IgAN与增殖性狼疮性肾小球肾炎的免疫组织学鉴别特别有用,增殖性狼疮性肾小球肾炎是在IgA免疫染色方面与IgAN重叠最大的肾小球肾炎形式。当IgAN的诊断标准为系膜区IgA为主或共显性免疫染色2+或更高且C1q免疫染色小于2+时,IgAN的免疫组织学诊断准确率为98%。

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