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快速进展性膜性肾病

Membranous Nephropathy with Rapid Progression.

作者信息

Vairakkani R, Valavan K Thirumal, Fernando M Edwin, Raj T Yashwanth

机构信息

Department of Nephrology, Government Stanley Medical College and Hospital, Old Jail Road, Chennai, Tamil Nadu, India.

出版信息

Indian J Nephrol. 2020 Jan-Feb;30(1):47-49. doi: 10.4103/ijn.IJN_85_19. Epub 2019 Dec 27.

Abstract

We report a 49-year-old man with microscopic hematuria, subnephrotic proteinuria, and rapidly progressive renal failure. His biopsy had features of PhosphoLipase A2 Receptor (PLA2R) positive membranous nephropathy with circumferential cellular crescents. Further work-up revealed IgG antiGlomerular Basement Membrane (anti-GBM) antibody titer of 188 U/mL (normal <7 U/mL). A final diagnosis of membranous nephropathy with anti-GBM disease was made. These two distinct pathological entities can occur together resulting in significant morbidity and mortality unless diagnosed early and treatment initiated promptly. Outcomes have been poor, given the nonspecific presentation and delay in diagnosis.

摘要

我们报告了一名49岁男性,患有镜下血尿、亚肾病范围蛋白尿和快速进展性肾衰竭。他的活检显示磷脂酶A2受体(PLA2R)阳性膜性肾病伴有环周细胞性新月体的特征。进一步检查发现IgG抗肾小球基底膜(抗GBM)抗体滴度为188 U/mL(正常<7 U/mL)。最终诊断为膜性肾病合并抗GBM病。这两种不同的病理实体可同时出现,除非早期诊断并及时开始治疗,否则会导致显著的发病率和死亡率。鉴于临床表现不特异且诊断延迟,预后较差。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7023/6977376/625ff894a7ff/IJN-30-47-g001.jpg

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