Jen Kuang-Yu, Auron Ari
Department of Pathology and Laboratory Medicine, University of California, Davis School of Medicine, Sacramento, CA, USA.
Department of Pediatric Nephrology, The Permanente Medical Group, Roseville, CA, USA.
Case Rep Nephrol. 2021 Jul 17;2021:2586693. doi: 10.1155/2021/2586693. eCollection 2021.
Classic antiglomerular basement membrane (anti-GBM) disease is an exceedingly rare but extremely aggressive form of glomerulonephritis, typically caused by autoantibodies directed against cryptic, conformational epitopes within the noncollagenous domain of the type IV collagen alpha-3 subunit. Pathologic diagnosis is established by the presence of strong, diffuse, linear staining for immunoglobulin on immunofluorescence microscopy. Recently, patients with atypical clinical and pathologic findings of anti-GBM disease have been described. These patients tend to have an indolent clinical course, without pulmonary involvement, and laboratory testing rarely reveals the presence of anti-GBM antibodies. Specific guidelines for the treatment and management of these patients are unclear. Here, we describe a case of atypical anti-GBM disease in a young child who presented with hematuria and prominent proteinuria. Throughout the course of his illness, creatinine remained normal. He was conservatively treated with steroids and rituximab, resulting in resolution of his clinical symptoms and normalization of laboratory findings.
经典抗肾小球基底膜（anti-GBM）病是一种极其罕见但极具侵袭性的肾小球肾炎，通常由针对IV型胶原α-3亚基非胶原结构域内隐蔽性构象表位的自身抗体引起。病理诊断通过免疫荧光显微镜检查发现免疫球蛋白呈强、弥漫性线性染色来确立。最近，已有抗GBM病非典型临床和病理表现患者的报道。这些患者往往临床病程进展缓慢，无肺部受累，实验室检查很少发现抗GBM抗体。这些患者的治疗和管理的具体指南尚不清楚。在此，我们描述一例患有血尿和显著蛋白尿的幼儿非典型抗GBM病病例。在其整个病程中，肌酐水平保持正常。他接受了类固醇和利妥昔单抗的保守治疗，临床症状得以缓解，实验室检查结果恢复正常。
