Abstract:
Antibrush border antibody (ABBA) disease is a rare cause of kidney disease characterized by progressive renal tubular injury associated with immune complex deposition along the basement membranes of the proximal tubule and circulating autoantibodies to brush border antigens. Several antigens have been identified as targets of autoantibodies in this disease, including low-density lipoprotein receptor related protein 2 (LRP2), cubilin, and amnionless proteins. We present 9 patients from 2 academic medical centers and describe the clinicopathologic characteristics and outcome data. All patients presented with acute kidney injury and proteinuria. Pathology confirmed immune complex deposition along proximal tubular basement membranes in all patients, but the majority (6/8) also showed segmental glomerular subepithelial immune complexes. Two of 3 patients treated with rituximab demonstrated stabilization of kidney function; 1 of these patients had mantle cell lymphoma. One patient with lung cancer showed stabilization of disease after treatment of the malignancy. The remaining patients progressed to end-stage kidney disease with either conservative therapy (3 patients) or immunosuppression with glucocorticoids (2 patients). This series highlights the poor prognosis of ABBA disease, but a potential benefit of anti-B cell therapy or treatment of an underlying malignancy in some cases.
摘要:
抗刷缘抗体(ABBA)病是肾脏疾病的罕见原因,其特征是进行性肾小管损伤与沿近端小管基底膜的免疫复合物沉积和针对刷缘抗原的循环自身抗体有关。几种抗原已被确定为该疾病中自身抗体的靶标,包括低密度脂蛋白受体相关蛋白2(LRP2),cubilin,和无羊膜蛋白质.我们介绍了来自2个学术医疗中心的9例患者,并描述了临床病理特征和结果数据。所有患者均表现为急性肾损伤和蛋白尿。病理学证实所有患者的免疫复合物沿近端肾小管基底膜沉积,但大多数(6/8)也显示节段性肾小球上皮下免疫复合物。3例接受利妥昔单抗治疗的患者中有2例表现出肾功能稳定;这些患者中有1例患有套细胞淋巴瘤。一名肺癌患者在治疗恶性肿瘤后显示疾病稳定。其余患者通过保守治疗(3例)或糖皮质激素免疫抑制(2例)进展为终末期肾脏疾病。该系列突出了ABBA疾病的不良预后,但在某些情况下,抗B细胞治疗或治疗潜在恶性肿瘤的潜在益处。