Anti-brush border antibody (ABBA)-associated renal disease.
Anti-brush border antibody (ABBA)-associated renal disease.
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抗刷状缘抗体（ABBA）相关的肾脏疾病。
DOI：
10.1093/qjmed/hcaa015
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发表时间：
2020
期刊：
影响因子：
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通讯作者：
Sekar,A
中科院分区：
文献类型：
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作者：
Campbell,RE;Uhlenhopp,D;Shaw,M;Dai,D-F;Sekar,A
讨论ABBA肾病通常发生在60岁以上的男性，他们表现为急性肾损伤(AKI)和肾下性蛋白尿，通常进展迅速到ESKD。1、2疾病过程被认为是由于产生了抗LRP2的抗体，并与C3一起以免疫复合体的形式沉积在PCT的TBM内，在较小程度上沉积在肾小球的上皮下膜。1，2 LRP2正常功能是重新吸收白蛋白和PCT内的其他蛋白质，是系统性自身免疫性疾病(如类风湿性关节炎)的主要自身抗原。4到目前为止，只报告了11例ABBA肾病，这是2016年的第一次报告。1-3以前报告的ABBA患者的人口统计学特征与恶性肿瘤的存在没有任何相关性。然而，有报道称，克隆氏病患者的血清中存在阿巴斯。5 ABBA病患者的肾活检显示广泛的肾小管损害，电子显微镜显示沿TBM和GBM有免疫复合体沉积。免疫荧光显示这些沉积物的免疫球蛋白和抗LRP2/megalin呈阳性。最佳治疗方案仍不清楚。对10名患者的回顾分析指出，只有一名患者在接受泼尼松和环磷酰胺治疗后血清学缓解。1接受观察、利妥昔单抗或强的松治疗的其他患者有不同的结果；5例需要肾脏替代治疗。1例患者接受了移植，并复发。1、2我们的病例突出了一种罕见但重要的严重进展性AKI的原因。需要未来的工作来确定最佳的检测方法和治疗方案。
DiscussionABBA renal disease typically occurs in males over the age of 60, who present with acute kidney injury (AKI) and subnephrotic proteinuria, and typically progress rapidly to ESKD. 1, 2 The disease process is thought to occur from IgG antibodies developing against anti-LRP2 and, together with C3, deposit as immune complexes within the TBM of the PCT, and to a lesser degree, the subepithelial membrane of the glomerulus. 1, 2 LRP2 normally functions to reabsorb albumin and other proteins within the PCT and is a major autoantigen in systemic autoimmune diseases, such as rheumatoid arthritis. 4 To date, only 11 cases of ABBA renal disease have been reported, the first report in 2016. 1–3 The demographics of patients previously reported to have ABBA have not shown any correlation to the presence of malignancy. However, there are reports of ABBAs present in the sera of patients with Crohn’s disease. 5 Renal biopsies in patients with ABBA disease show extensive tubular damage, with electron microscopy highlighting immune complex deposits along the TBM and GBM. These deposits stain positive for IgG and anti-LRP2/megalin via immunofluorescence. Optimal treatment remains unclear. A retrospective analysis of 10 patients noted that only one patient had serologic remission following treatment with prednisone and cyclophosphamide. 1 The other patients that were treated with observation, rituximab or prednisone had various outcomes; five required renal replacement therapy. 1 One patient received a transplant and had recurrence of the disease. 1, 2 Our case highlights a rare, yet important, cause of severe and progressive AKI. Future work is needed to determine the best detection methods and treatment options.