关键词: Antineutrophil cytoplasmic autoantibody Case report Pauci-immune crescentic glomerulonephritis Systemic lupus erythematosus

来  源:   DOI:10.1159/000527248   PDF(Pubmed)

Abstract:
Renal involvement in systemic lupus erythematosus is usually exhibited as lupus nephritis, which is a form of immune complex-mediated glomerulonephritis and one of the most severe organ manifestations of systemic lupus erythematosus. The pathogenesis involved glomerular immune complex deposition, which leads to glomerular inflammation and typically shows a \"full-house\" pattern on immunofluorescence microscopy. Other forms of glomerulonephritis are rarely observed in patients with systemic lupus erythematosus. Pauci-immune crescentic glomerulonephritis is the pattern of injury most commonly observed in patients with antineutrophil cytoplasmic autoantibody-associated glomerulonephritis. The characteristic histological feature of pauci-immune crescentic glomerulonephritis is focal necrotizing and crescentic glomerulonephritis with little or no glomerular staining for immunoglobulin by immunofluorescence microscopy. We report a rare case of antineutrophil cytoplasmic autoantibody-negative pauci-immune crescentic glomerulonephritis in a patient with systemic lupus erythematosus.
摘要:
系统性红斑狼疮的肾脏受累通常表现为狼疮性肾炎,这是一种免疫复合物介导的肾小球肾炎,也是系统性红斑狼疮最严重的器官表现之一。发病机制涉及肾小球免疫复合物沉积,导致肾小球炎症,通常在免疫荧光显微镜上显示出“全屋”模式。在系统性红斑狼疮患者中很少观察到其他形式的肾小球肾炎。免疫缺陷性新月体肾小球肾炎是在抗中性粒细胞胞浆自身抗体相关性肾小球肾炎患者中最常见的损伤模式。免疫缺陷性新月体性肾小球肾炎的特征性组织学特征是局灶性坏死性和新月体性肾小球肾炎,免疫荧光显微镜对肾小球免疫球蛋白染色很少或没有染色。我们报告了一例罕见的系统性红斑狼疮患者的抗中性粒细胞胞浆自身抗体阴性的保护性新月体性肾小球肾炎。
公众号