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伴有满堂亮肾病的抗中性粒细胞胞浆抗体阳性血管炎:一种不寻常的关联——病例报告及文献复习

Anca-positive vasculitis with full-house nephropathy, an unusual association: a case report and review of literature.

作者信息

Montalvo Carlos Mauricio Martínez, Gutierrez Laura Catalina, Perez Carolina, Delgado Harrison Herrera, Barrios Paula Corinna Martinez

机构信息

Universidad del Rosario, Bogota, Colombia.

Universidad Nuestra Señora Del Rosario, Bogota, Colombia.

出版信息

J Bras Nefrol. 2022 Apr-Jun;44(2):285-290. doi: 10.1590/2175-8239-JBN-2020-0134.

Abstract

Rapidly progressive glomerulonephritis is a medical emergency, with mortality around 20%. It is characterized by crescent glomerulonephritis and progressive loss of kidney function, hematuria, and proteinuria. Its classification is given by immunofluorescence detection of antibodies against glomerular basement membrane (Anti-MBG), immunocomplexes, or pauci-immune pattern. Its etiology should be based on clinical findings, immunological profile, age, sex, and histopathological characteristics. We present a case of a 27-year-old woman with symptoms consistent with rapidly progressive glomerulonephritis and biopsy findings of a full-house kidney nephropathy, with an early fatal outcome. An association of low incidence, as it is a case with a full-house pattern, and an autoimmune profile for negative systemic lupus erythematosus makes this a rare case. ANCA-associated vasculitis with full-house kidney disease was diagnosed, an unusual condition with up to 3% presentation and few reports in the literature, highlighting the importance of its reporting and contribution to the literature.

摘要

急进性肾小球肾炎是一种医疗急症,死亡率约为20%。其特征为新月体性肾小球肾炎以及肾功能进行性丧失、血尿和蛋白尿。通过免疫荧光检测抗肾小球基底膜抗体(抗MBG)、免疫复合物或寡免疫模式进行分类。其病因应基于临床症状、免疫特征、年龄、性别和组织病理学特征。我们报告一例27岁女性病例,其症状符合急进性肾小球肾炎,活检结果为满堂亮肾病,早期预后不良。由于该病例为满堂亮模式且系统性红斑狼疮自身免疫特征为阴性,这种关联发病率较低,使其成为罕见病例。诊断为抗中性粒细胞胞浆抗体相关性血管炎伴满堂亮肾病,这是一种不常见的疾病,发病率高达3%,文献报道较少,凸显了报告该病例并为文献做出贡献的重要性。

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