The renal pathology of primary antiphospholipid syndrome: a distinctive form of endothelial injury
The renal pathology of primary antiphospholipid syndrome: a distinctive form of endothelial injury
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DOI：
10.1093/qjmed/93.7.457
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发表时间：
2000-07-01
影响因子：
13.3
通讯作者：
Neild, GH
中科院分区：
文献类型：
--
作者：
Griffiths, MH;Papadaki, L;Neild, GH
原发性抗磷脂综合征（APS）的血管和肾小球病理学的一些特点是公认的，但我们描述了新的肾小球超微结构的变化，我们认为是APS的特异性。对8例APS患者的肾活检进行了光镜和电镜检查。所有患者均有抗心磷脂抗体，临床表现从暴发性多系统疾病到孤立性蛋白尿不等。光镜下六胺银染色显示肾小球基底膜的重叠和复制。通过电子显微镜，多余的，褶皱的基底膜段伴随着一个“新的”更直的薄基底膜邻近内皮。在2例病例中，临床上未怀疑这些抗体的存在，并且没有血栓性微血管病的临床病史或证据。我们描述了一个独特的肾小球病变，代表了一种无法解释的内皮损伤形式在这种综合征。
Some features of the vascular and glomerular pathology of primary antiphospholipid syndrome (APS) are well recognized, but we describe novel glomerular ultrastructural changes that we consider to be pathognomonic of APS. Renal biopsies from eight patients with APS were examined by light and electron microscopy. All had anti-cardiolipin antibodies, and the clinical presentation ranged from fulminant multi-system disease to isolated proteinuria. By light microscopy, the hexamine silver stain showed a combination of glomerular basement membrane wrinkling and reduplication. By electron microscopy, redundant, wrinkled segments of basement membrane were accompanied by a 'new' straighter thin basement membrane adjacent to the endothelium. In two cases the presence of these antibodies was not suspected clinically, and there was no clinical history or evidence of a thrombotic microangiopathy. We describe a distinctive glomerular lesion that represents an unexplained form of endothelial injury in this syndrome.