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Abstract：
Anti-glomerular basement membrane (GBM) antibody nephritis is defined by linear immunofluorescence staining of GBM by immunoglobulin G (IgG), typically associated with GBM rupture, fibrinoid necrosis, and crescent formation. Clinically, the patients present with rapidly worsening renal function, often with hematuria. Typical renal pathologic findings include necrotizing and crescentic glomerulonephritis. In contrast, thrombotic microangiopathy (TMA) is characterized by microvascular thrombosis, which can also lead to acute kidney injury. Thrombotic microangiopathy is associated with some systemic diseases and has characteristic clinical features of microangiopathic hemolytic anemia, platelet consumption, and multiple organ failure. Anti-GBM nephritis associated with TMA has rarely been reported. We describe an unusual case of atypical anti-GBM disease without crescent formation or necrosis but with light microscopic and ultrastructural features consistent with endothelial cell injury and glomerular-limited TMA.
摘要：
抗肾小球基底膜（GBM）抗体肾炎通过免疫球蛋白G（IgG）对GBM进行线性免疫荧光染色来定义，通常与GBM破裂有关，纤维蛋白样坏死，和新月形成。临床上,肾功能迅速恶化的患者，常伴有血尿。典型的肾脏病理表现包括坏死性和新月体肾小球肾炎。相比之下,血栓性微血管病（TMA）的特征是微血管血栓形成，这也可能导致急性肾损伤。血栓性微血管病与一些系统性疾病相关，具有微血管病性溶血性贫血的特征性临床特征。血小板消耗,多器官衰竭.很少报道与TMA相关的抗GBM肾炎。我们描述了一种不寻常的非典型抗GBM疾病，没有新月形成或坏死，但具有与内皮细胞损伤和肾小球受限TMA一致的光学显微镜和超微结构特征。
