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移植肾中反复发作的非典型抗肾小球基底膜肾炎。

Recurrent atypical antiglomerular basement membrane nephritis in the kidney transplant.

机构信息

Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.

Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, Missouri, USA.

出版信息

Am J Transplant. 2024 Jan;24(1):123-133. doi: 10.1016/j.ajt.2023.09.007. Epub 2023 Sep 27.

Abstract

Atypical antiglomerular basement membrane (anti-GBM) nephritis can be defined as linear GBM staining for monotypic or polytypic immunoglobulin (Ig) by immunofluorescence (IF) without a diffuse crescentic pattern. We describe the clinicopathologic features of 6 patients (18 biopsies) in this first series of recurrent atypical anti-GBM nephritis after kidney transplantation. Recurrent glomerulonephritis occurred at a mean of 3.8 months posttransplant (range 1-7 months). Three index biopsies were for clinical indication, and 3 were protocol biopsies. Glomerular histologic changes were mild, with 2 showing segmental endocapillary hypercellularity, 1 focal glomerular microangiopathy, and the others no significant glomerular histologic changes. All 6 allografts showed monotypic linear glomerular Ig staining by IF: IgG kappa (n = 2), IgG lambda, IgA kappa, IgA lambda, and IgM lambda. Follow-up biopsies were available for 5 patients and showed similar histologic and IF findings without evidence of significant progression. No patients had detectable serum anti-GBM antibody or monoclonal proteins. The mean serum creatinine level on follow-up (24-62 months posttransplant) was 1.8 (range 0.93-2.77) mg/dL; no grafts were lost to recurrent disease. This series demonstrates that monotypic atypical anti-GBM recurs in the allograft and supports the idea that this disease is due to a circulating monoclonal protein.

摘要

非典型抗肾小球基底膜 (anti-GBM) 肾炎可定义为免疫荧光 (IF) 显示线性 GBM 单克隆或多克隆免疫球蛋白 (Ig) 染色,而无弥漫性新月体模式。我们描述了 6 例(18 个活检)首例复发性肾移植后非典型抗 GBM 肾炎的临床病理特征。复发性肾小球肾炎发生在移植后平均 3.8 个月(范围 1-7 个月)。3 个索引活检是临床指征,3 个是协议活检。肾小球组织学变化轻微,2 例表现为节段性毛细血管内细胞增多症,1 例为局灶性肾小球微血管病,其余无明显肾小球组织学变化。所有 6 例同种异体移植物均通过 IF 显示单克隆线性肾小球 Ig 染色:IgG kappa(n = 2)、IgG lambda、IgA kappa、IgA lambda 和 IgM lambda。5 例患者可获得随访活检,显示相似的组织学和 IF 发现,无明显进展证据。没有患者检测到血清抗 GBM 抗体或单克隆蛋白。移植后随访(移植后 24-62 个月)的平均血清肌酐水平为 1.8(范围 0.93-2.77)mg/dL;没有因复发性疾病而丧失移植物。该系列表明单克隆非典型抗 GBM 在同种异体移植物中复发,并支持该疾病是由循环单克隆蛋白引起的观点。

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