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增生性狼疮性肾炎相关性肾小球病:可逆转的急性肾损伤。

Collapsing glomerulopathy associated with proliferative lupus nephritis: reversible acute kidney injury.

机构信息

Nephrology Division, University of Sao Paulo School of Medicine, Sao Paulo, Brazil.

出版信息

Lupus. 2011 Jan;20(1):98-101. doi: 10.1177/0961203310381516. Epub 2010 Nov 15.

Abstract

Collapsing glomerulopathy is a rare form of glomerular injury, characterized by segmental or global collapse of the glomerular capillaries, wrinkling and retraction of the glomerular basement membrane, and marked hypertrophy and hyperplasia of podocytes. Prognosis is usually poor, with most cases developing end-stage renal disease, in spite of treatment. The association of collapsing glomerulopathy and systemic lupus erythematosus is very unusual. In this report, we describe the first case of a simultaneous diagnosis of collapsing glomerulopathy and diffuse proliferative lupus nephritis. The case presented with acute kidney injury and nephrotic syndrome and evolved with partial remission of nephrotic syndrome and recovery of renal function after aggressive treatment with intravenous cyclophosphamide and methylprednisolone.

摘要

塌陷性肾小球病是一种罕见的肾小球损伤形式,其特征是肾小球毛细血管节段性或全球性塌陷、肾小球基底膜起皱和回缩,以及足细胞显著肥大和增生。尽管进行了治疗,但预后通常较差,大多数病例会发展为终末期肾病。塌陷性肾小球病与系统性红斑狼疮的关联非常罕见。在本报告中,我们描述了首例同时诊断为塌陷性肾小球病和弥漫性增生性狼疮性肾炎的病例。该病例表现为急性肾损伤和肾病综合征,并在接受静脉注射环磷酰胺和甲基强的松龙的强化治疗后,部分缓解了肾病综合征并恢复了肾功能。

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