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狼疮性血管病致肾结局良好 1 例。

A case of lupus vasculopathy presenting favorable renal outcome.

机构信息

Department of Nephrology, University of Miyazaki Hospital, Faculty of Medicine, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki, 889-1692, Japan.

Department of Rheumatology and Infectious Diseases, University of Miyazaki Hospital, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan.

出版信息

CEN Case Rep. 2020 Feb;9(1):74-80. doi: 10.1007/s13730-019-00431-2. Epub 2019 Dec 20.

Abstract

Noninflammatory necrotizing vasculopathy, also referred to as lupus vasculopathy, is not infrequently observed in the pathology of lupus nephritis. It affects vessels causing them to become severely narrowed and occluded by a mechanism involving immune complexes. We experienced a 51-year-old woman with lupus nephritis class IV + V, which was accompanied by lupus vasculopathy. Renal biopsy and light microscopy showed eosinophilic hyaline-like material in the afferent and/or efferent arterioles, which narrowed the lumen, and which were positive for IgG by immunofluorescent analysis. Electron microscopy indicated that amorphous material and endothelial detachment occluded the arterioles. These findings were consistent with those of lupus vasculopathy. We treated the patient with steroids and cyclophosphamide. By the day of discharge, her levels of creatinine and proteinuria had undergone partial remission. Although lupus vasculopathy was implied as a lesion with unfavorable renal prognosis, some recent reports suggest its true renal prognosis is not unfavorable necessarily. Nevertheless, lupus vasculopathy is an important finding in diagnosis in contradiction to other vascular legions in systemic lupus erythematosus. In addition, a standard therapy has also not been established. Therefore, it is important to accumulate cases of lupus vasculopathy to determine its prognosis and develop standard treatments.

摘要

非炎症性坏死性血管病变,也称为狼疮性血管病,在狼疮肾炎的病理学中经常观察到。它影响血管,导致免疫复合物机制导致血管严重狭窄和闭塞。我们遇到一位 51 岁的狼疮肾炎 IV+V 级女性患者,同时伴有狼疮性血管病。肾活检和光镜检查显示,入球小动脉和/或出球小动脉中存在嗜酸性玻璃样物质,管腔变窄,免疫荧光分析显示 IgG 阳性。电子显微镜显示无定形物质和内皮脱落阻塞了小动脉。这些发现与狼疮性血管病一致。我们用类固醇和环磷酰胺治疗了患者。出院当天,她的肌酐和蛋白尿水平部分缓解。虽然狼疮性血管病被暗示为一种对肾脏预后不利的病变,但最近的一些报告表明其真正的肾脏预后不一定不利。然而,狼疮性血管病是系统性红斑狼疮中与其他血管病变相区别的重要诊断发现。此外,也尚未建立标准治疗方法。因此,重要的是要积累狼疮性血管病的病例,以确定其预后并制定标准治疗方法。

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A case of lupus vasculopathy presenting favorable renal outcome.狼疮性血管病致肾结局良好 1 例。
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