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