Montalvo Carlos Mauricio Martínez, Gutierrez Laura Catalina, Perez Carolina, Delgado Harrison Herrera, Barrios Paula Corinna Martinez
Universidad del Rosario, Bogota, Colombia.
Universidad Nuestra Señora Del Rosario, Bogota, Colombia.
J Bras Nefrol. 2022 Apr-Jun;44(2):285-290. doi: 10.1590/2175-8239-JBN-2020-0134.
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.
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。