Department of Hematology, CHU Rennes, Rennes, France.
Department of Pathology, CHU Rennes, Rennes, France.
BMC Nephrol. 2019 Jan 8;20(1):6. doi: 10.1186/s12882-018-1197-6.
Goodpasture Syndrome (GS) is an autoimmune disease caused by the development of auto-antibodies against the Glomerular Basement Membrane (GBM). Linear deposit of immunoglobulins G on the GBM detected by immunofluorescence analysis of renal biopsies is a GS pathognomonic finding. GS is commonly monophasic and its incidence is 1.6 case per million per year.
This report describes and discusses the case of a 40-year-old woman who one year after allograft kidney transplant, presented with acute pulmonary and renal symptoms of GS, leading to acute graft dysfunction, without circulating anti-GBM antibody detection in laboratory assays. She received a living donor kidney transplant 4 years after the first diagnosis of GS without circulating anti-GBM antibodies, when considered in remission.
In both episodes, the diagnosis of GS was based exclusively on the kidney biopsy that showed rapidly progressing glomerulonephritis with deposition of immunoglobulins G on the GBM. Although rare, the management of patients with GS without circulating anti-GBM antibodies is difficult due to the lack of standardized follow-up guidelines to reduce the risk of GS recurrence after kidney transplantation.
Goodpasture 综合征(GS)是一种由针对肾小球基底膜(GBM)的自身抗体引起的自身免疫性疾病。通过对肾活检的免疫荧光分析检测到 GBM 上线性沉积的免疫球蛋白 G 是 GS 的特征性发现。GS 通常是单相的,其发病率为每年每百万人口 1.6 例。
本报告描述并讨论了一位 40 岁女性的病例,她在肾移植后一年出现了 GS 的急性肺部和肾脏症状,导致急性移植物功能障碍,实验室检测未检测到循环抗 GBM 抗体。她在首次诊断为 GS 后 4 年接受了活体供肾移植,当时被认为处于缓解期,且未检测到循环抗 GBM 抗体。
在这两个发病阶段,GS 的诊断均仅基于肾活检,活检显示快速进展性肾小球肾炎,GBM 上有免疫球蛋白 G 沉积。尽管罕见,但由于缺乏标准化的随访指南来降低肾移植后 GS 复发的风险,因此管理无循环抗 GBM 抗体的 GS 患者具有一定难度。