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合并2级和3级狼疮性肾炎的塌陷型局灶节段性肾小球硬化:1例病例报告及文献复习

Collapsing FSGS with Concurrent Class 2 and 3 Lupus Nephritis: A Case Report and Review of the Literature.

作者信息

Vanood Aimen, Owen Ryan, Maraskine Marina, Schreiber Ariyon, Pokharel Rajesh, Cohen Lisa

机构信息

Oakland University William Beaumont School of Medicine, Rochester, Michigan, USA.

Department of Internal Medicine, Beaumont Health System, Royal Oak, Michigan, USA.

出版信息

Case Rep Nephrol Dial. 2021 Jan 28;11(1):16-25. doi: 10.1159/000510840. eCollection 2021 Jan-Apr.

Abstract

Lupus nephritis (LN) and the collapsing variant of focal segmental glomerulosclerosis (cFSGS) are separate histologic diagnoses that are generally thought to have separate etiologies. We describe the presentation of a 20-year-old African American female with advanced renal failure (creatinine 7.16 mg/dL), nephrotic-range proteinuria, and a 30-pound weight loss. Renal biopsy demonstrated class 2 and 3 LN as well as cFSGS. A review of the current literature demonstrates that the dual diagnosis of LN and cFSGS may not be as rare as previously understood. Whether the presence of one of these pathophysiologic processes predisposes a patient to the development of the other, or whether genetic variation increases the risk for development of both conditions, remains unclear. Currently there is no standard therapy to manage these patients, and overall renal prognosis is poor.

摘要

狼疮性肾炎(LN)和局灶节段性肾小球硬化的塌陷型(cFSGS)是不同的组织学诊断,通常认为它们有不同的病因。我们描述了一名20岁非裔美国女性的病例,她患有晚期肾衰竭(肌酐7.16mg/dL)、肾病范围蛋白尿以及体重减轻30磅。肾活检显示为2级和3级LN以及cFSGS。对当前文献的回顾表明,LN和cFSGS的双重诊断可能并不像之前认为的那么罕见。这两种病理生理过程中的一种是否会使患者易患另一种疾病,或者基因变异是否会增加患这两种疾病的风险,目前尚不清楚。目前尚无标准疗法来治疗这些患者,总体肾脏预后较差。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a0dc/7923702/b6600a68c11f/cnd-0011-0016-g01.jpg

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