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一名青少年的快速进展性狼疮性肾小球肾炎及伴发的微血管病

Rapidly progressive lupus glomerulonephritis and concomitant microangiopathy in an adolescent.

作者信息

Blake J S, Butani L

出版信息

Lupus. 2002;11(8):533-5. doi: 10.1191/0961203302lu227cr.

Abstract

We describe our experience managing a 16-year-old girl with systemic lupus erythematosus (SLE) who presented concomitantly with rapidly progressive glomerulonephritis (RPGN) and a thrombotic microangiopathic hemolytic anemia (TMAHA). Her renal biopsy showed evidence of diffuse proliferative glomerulonephritis without glomerular microthrombi. The patient was treated with a combination of intravenous corticosteroids and cyclophosphamide, as well as plasmapheresis, with an excellent response resulting in complete disease remission. The purpose of our report is to make health professionals more aware of TMAHA as a complication of SLE, since the occurrence of TMAHA may confuse the clinical picture, and since its treatment with plasmapheresis is life saving, if performed early.

摘要

我们描述了我们对一名16岁系统性红斑狼疮(SLE)女孩的治疗经验,该女孩同时出现快速进展性肾小球肾炎(RPGN)和血栓性微血管病性溶血性贫血(TMAHA)。她的肾活检显示有弥漫性增殖性肾小球肾炎的证据,但无肾小球微血栓形成。该患者接受了静脉注射皮质类固醇和环磷酰胺联合治疗以及血浆置换,反应良好,疾病完全缓解。我们报告的目的是让医疗专业人员更加了解TMAHA作为SLE的一种并发症,因为TMAHA的出现可能会使临床情况变得复杂,而且如果早期进行血浆置换治疗,它是可以挽救生命的。

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