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一名狼疮性肾炎年轻患者出现急性肾损伤、反复癫痫发作和血小板减少症:诊断难题

Acute Kidney Injury, Recurrent Seizures, and Thrombocytopenia in a Young Patient with Lupus Nephritis: A Diagnostic Dilemma.

作者信息

Alvarado Verduzco Hector, Acharya Anjali

机构信息

Department of Internal Medicine, Division of Internal Medicine, Jacobi Medical Center, Bronx, NY, USA.

Department of Nephrology, Division of Internal Medicine, Jacobi Medical Center, Bronx, NY, USA.

出版信息

Case Rep Nephrol. 2016;2016:7104098. doi: 10.1155/2016/7104098. Epub 2016 Dec 4.

Abstract

. Posterior reversible encephalopathy syndrome (PRES) is a constellation of clinical and radiologic findings. Fluctuations in blood pressure, seizures, and reversible brain MRI findings mainly in posterior cerebral white matter are the main manifestations. PRES has been associated with multiple conditions such as autoimmune disorders, pregnancy, organ transplant, and thrombotic microangiopathy (TMA). . A 22-year-old woman with history of Systemic Lupus Erythematous complicated with chronic kidney disease secondary to lupus nephritis class IV presented with recurrent seizures and uncontrolled hypertension. She was found to have acute kidney injury and thrombocytopenia. Repeat kidney biopsy showed diffuse endocapillary and extracapillary proliferative and membranous lupus nephritis (ISN-RPS class IV-G+V) and endothelial swelling secondary to severe hypertension but no evidence of TMA. Brain MRI showed reversible left frontal and parietal lesions that resolved after controlling the blood pressure, making PRES the diagnosis. . PRES is an important entity that must be recognized and treated early due to the potential reversibility in the early stages. Physicians must have high suspicion for these unusual presentations. We present a case where performing kidney biopsy clinched the diagnosis in our patient with multiple confounding factors.

摘要

后部可逆性脑病综合征(PRES)是一组临床和影像学表现。血压波动、癫痫发作以及主要位于大脑后部白质的可逆性脑MRI表现是其主要症状。PRES与多种疾病相关,如自身免疫性疾病、妊娠、器官移植和血栓性微血管病(TMA)。一名22岁女性,有系统性红斑狼疮病史,并发狼疮性肾炎IV级继发的慢性肾脏病,出现反复癫痫发作和难以控制的高血压。她被发现有急性肾损伤和血小板减少。重复肾活检显示弥漫性毛细血管内和毛细血管外增生性及膜性狼疮性肾炎(国际肾脏病学会/肾脏病理学会分类IV - G + V)以及严重高血压继发内皮肿胀,但无TMA证据。脑部MRI显示可逆性左侧额叶和顶叶病变,血压控制后病变消退,诊断为PRES。PRES是一个重要的疾病实体,因其早期具有潜在可逆性,必须尽早识别和治疗。医生必须对这些不寻常的表现高度怀疑。我们报告一例病例:在存在多种混淆因素的患者中,进行肾活检明确了诊断。

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