DENSE DEPOSIT DISEASE - VARIANT OF MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS
DENSE DEPOSIT DISEASE - VARIANT OF MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS
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DOI：
10.1038/ki.1975.32
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发表时间：
1975-01-01
影响因子：
19.6
通讯作者：
LEVY, M
中科院分区：
文献类型：
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作者：
HABIB, R;GUBLER, MC;LEVY, M
在先前的研究中已经证明，膜增生性肾小球肾炎（MPGN）有几种形态学变体[1-3]。在大多数情况下，系膜细胞增殖和系膜基质增加与毛细血管壁增厚有关（“经典MPGN”）。在某些病例中，除了先前的发现外，由于大多数小叶中心区存在结节，肾小球丛分叶加重。这伴随着毛细血管腔的外周移位或闭塞（小叶GN或“小叶型MPGN”）。然而，通过光镜和电镜对毛细血管壁增厚的分析揭示了两种类型的参与：第一种，毛细血管壁增厚是由于系膜基质插入内皮细胞和正常基底膜之间，产生“双轮廓”外观。电子显微镜检查以及免疫荧光显微镜检查显示，在所有情况下，存在异常的内皮下存款。这种类型的肾小球肾炎被称为伴有内皮下沉积物（SED）。在第二种情况下，毛细血管壁的增厚是由于基底膜本身存在异常致密的亲水性物质。“双等高线”在这里是一个不稳定的发现。这种类型的肾小球肾炎被命名为膜内致密沉积（DIMD），最近又被称为板层肾小球肾炎[4]。目前，在大多数临床研究中，这两种类型的肾小球肾炎并不能相互区分，因为在光学显微镜下很难识别MPGN和DIMD。因此，除了一个例外[5]，没有广泛的研究涉及这种特殊的实体。我们观察了44例这种类型的MPGN，并在此报告了详细的临床和补充研究，以及这些病例的组织学数据，以与同期观察到的84例MPGN伴SED进行比较。
It has been demonstrated in previous studies that there are several morphological variants of membranoproliferative glomerulonephritis (MPGN) [1–3]. In most cases mesangial cell proliferation and an increase in mesangial matrix are associated with capillary wall thickening (“classical MPGN”). In some cases, in addition to the previous findings, there is an accentuation of lobulation of glomerular tufts due to the presence of sclerotic nodules in most of the centrilobular areas. This is accompanied by peripheral displacement or obliteration of capillary lumens (lobular GN or “MPGN with lobular pattern”). More or less abundant epithelial crescents may be seen in both variants.However, analysis of the capillary wall thickening by light and electron microscopy reveals two types of involvement: In the first, thickening of the capillary walls is due to an interposition of mesangial matrix between the endothelium and a normal basement membrane, producing a “double contour” appearance. Electron microscopy, as well as immunofluorescence microscopy, reveals in all cases the presence of abnormal subendothelial deposits. This variety is calledMPGN with subendothelial deposits (SED). In the second, the thickening of the capillary walls is due to the presence of an abnormal dense refractile material located in the basement membrane itself. “Double contours” here are an inconstant finding. This variety warrants the name ofMPGN with dense intramembranous deposits (DIMD); it has recently been calledlaminal glomerulonephritis [4].These two varieties of MPGN are at present not distinguished one from another in most clinical studies because it may be difficult to recognize MPGN with DIMD on light microscopy. Therefore, with one exception [5], there are no extensive studies dealing with this specific entity.We observed 44 cases of this variety of MPGN and report here detailed clinical and complement studies, together with histological data in these cases for comparison with 84 cases of MPGN with SED seen during the same period of time.