RECURRENCE OF MESANGIAL DEPOSITION OF IGA AFTER RENAL-TRANSPLANTATION
RECURRENCE OF MESANGIAL DEPOSITION OF IGA AFTER RENAL-TRANSPLANTATION
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DOI：
10.1038/ki.1975.35
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发表时间：
1975-01-01
影响因子：
19.6
通讯作者：
BARBANEL, C
中科院分区：
文献类型：
--
作者：
BERGER, J;YANEVA, H;BARBANEL, C
在法国约20%的特发性肾小球疾病成年患者中发现IgA系膜沉积。沉积累及每个肾小球的系膜，并在整个肾病过程中持续存在。因此，我们认为这是这些患者疾病的标志[1,2]。系膜IgA不含分泌片，常伴有一些IgG， IgM已被一些观察者发现[3,4]，但我们没有发现。C3在没有Clq或C4的情况下存在[4,5]，表明补体系统通过替代途径[6]激活，properdin的证明进一步支持了这一点[4,7]。然而，根据我们的经验，与急性肾小球肾炎或膜增殖性肾小球肾炎相比，properdin的沉积不那么明显。在光镜下，肾脏通常表现为局灶性增生性肾小球肾炎，但在薄切片和良好的三色染色下，即使没有局灶性改变的肾小球，也经常可以在系膜中看到一些纤维蛋白沉积。部分病例可见轻度弥漫性系膜细胞增多，有时灶性改变与局灶性肾小球硬化相似。在晚期病例中，间质和小动脉的改变变得明显，如果没有免疫荧光显微镜的帮助，即使是经验丰富的病理学家也可能误诊为间质性肾炎或肾硬化。在电子显微镜下，每个肾小球的系膜都可见电子致密沉积物。银浸渍可能是区分基底膜和系膜基质沉积物的必要条件。有时沉积物可轻微延伸至内皮下区域。系膜细胞的细胞器通常比平常丰富，但没有证据表明这些细胞吞噬沉积物。成人患者通常表现为轻蛋白尿和镜下血尿。在一半的病例中，肉眼可见的血尿要么是单次发作，要么是反复发作。肉眼血尿通常由上呼吸道感染或剧烈运动引起。可能伴有腰痛和排尿困难。有些病人蛋白尿较重，偶尔会出现肾病综合征。血尿和蛋白尿可能在不同的时间内消失，但在临床缓解期间获得的肾活检标本显示肾小球系膜沉积物持续存在。大多数成年患者的病程是长期的，但其临床表现是轻微的，几十年来几乎没有进展的证据。然而，高血压和肾功能不全可能发生。终末期肾衰竭最终发展为五分之一的病例，不幸的是，我们无法预测哪个病人会遵循这一过程。系膜沉积的数量与疾病的严重程度之间没有关系，在一个给定的病人中，系膜沉积通常保持相当恒定。在儿童肾小球疾病中，约有10%的肾小球疾病是由IgA的肾小球系膜沉积引起的。它几乎总是表现为复发性血尿[8,9]；肾组织在光镜下通常看起来正常。儿童复发性血尿的预后通常被认为是良好的，因此值得注意的是，本报告中有几例患者的疾病始于儿童时期。在大多数患者中，肾小球系膜IgA沉积，血清IgA浓度高，但在一些患者，可能是正常的，甚至低。血清IgG、IgM和…
Mesangial deposition of IgA is found in about 20% of adult patients with idiopathic glomerular disease in France. The deposition involves the mesangium of every glomerulus and persists during the whole course of the nephropathy. We therefore believe it to be the hallmark of the disease in these patients [1, 2].Mesangial IgA does not contain secretory piece, and it is usually accompanied by some IgG, IgM has been found by some observers [3, 4], but not by ourselves. C3 is present without Clq or C4 [4, 5], suggesting activation of the complement system by the alternate pathway [6], This is further supported by the demonstration of properdin [4,7]. In our experience, however, the deposition of properdin is much less conspicuous than in acute glomerulonephritis or membranoproliferative glomerulonephritis.By light microscopy, the kidney usually has the appearance of focal proliferative glomerulonephritis but, with thin sections and good trichrome stains, it is often possible to see some fibrinoid deposits in the mesangium of even those glomeruli which show no focal changes. Mild diffuse mesangial hypercellularity is present in some cases, and sometimes the focal changes may be similar to those observed in focal glomerulosclerosis. In advanced cases, interstitial and arteriolar changes become prominent, and without the help of immunofluorescence microscopy, these cases may be misdiagnosed as interstitial nephritis or nephrosclerosis even by experienced pathologists.With the electron microscope, electron-dense deposits are seen in the mesangium of every glomerulus. Silver impregnation may be necessary to clearly distinguish the deposits from the basement membrane and the mesangial matrix. There may sometimes be a slight extension of the deposits to the subendothelial areas. The cytoplasm of mesangial cells is usually richer in organelles than usual but no evidence of phagocytosis of the deposits by these cells can be demonstrated.In adult patients, the disease is usually manifested by light proteinuria and microscopic hematuria. In one-half of the cases, macroscopic hematuria occurs either as a single episode or as recurrent attacks. Gross hematuria is usually precipitated by an upper respiratory tract infection or strenuous exercise. It may be accompanied by loin pain and dysuria. In some patients proteinuria is heavier, and occasionally the nephrotic syndrome may develop. Hematuria and proteinuria may disappear for variable periods of time, but renal biopsy specimens obtained during periods of clinical remission have shown that the mesangial deposits persist.The course of the disease in most adult patients is long-standing but its clinical expression is mild, with little evidence of progression over decades. Nevertheless, hypertension and renal insufficiency may occur. Terminal renal failure eventually develops in about one case in five, and unfortunately we know of no way to predict which patient will follow this course. There is no relationship between the amount of mesangial deposition, which usually remains fairly constant in a given patient, and the severity of the disease.In the child, the mesangial deposition of IgA accounts for about 10% of cases of glomerular disease. It nearly always presents as recurrent hematuria [8,9]; renal tissue often looks normal by light microscopy. The prognosis of recurrent hematuria in children is generally considered to be good, and it is therefore of note that the disease began during childhood in several patients of this report.In most patients, with mesangial deposition of IgA, the serum concentration of IgA is high, but in some patients, it may be normal or even low. The serum concentrations of IgG, IgM and …