Division of Kidney Diseases and Hypertension, Glomerular Center at Northwell Health, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, New York.
Department of Pathology, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, New York.
Adv Kidney Dis Health. 2024 Jul;31(4):374-383. doi: 10.1053/j.akdh.2024.03.006.
Fibrillary glomerulonephritis (FGN) is a rare kidney disease typically affecting individuals in middle age, frequently presenting with advanced renal failure, proteinuria, and hypertension. FGN can be associated with autoimmune diseases, hepatitis C infection, and malignancies. Its exact pathogenesis remains elusive, and the exact role of DnaJ homolog subfamily B member 9 is yet to be determined. On renal biopsy, FGN exhibits distinctive Congo-red-negative, nonbranching fibrils, approximately 20 nm in diameter. DnaJ homolog subfamily B member 9 immunohistochemical staining has become a gold standard for diagnosis. Atypical variants exist, including congophilic, monotypic, and crescentic FGN, highlighting the disease's heterogeneity. Treatment with immunosuppression, including rituximab, has shown variable success, with no standard therapy established. FGN often leads to end-stage kidney disease, with a median progression time of 2-4 years postdiagnosis. Kidney transplantation is a viable option for FGN-related end-stage kidney disease, but recurrence in transplanted kidneys is not rare.
纤维状肾小球肾炎（FGN）是一种罕见的肾脏疾病，通常影响中年人群，常表现为晚期肾衰竭、蛋白尿和高血压。FGN 可与自身免疫性疾病、丙型肝炎感染和恶性肿瘤相关。其确切的发病机制仍不清楚，DnaJ 同源物亚家族 B 成员 9 的确切作用仍有待确定。在肾活检中，FGN 表现为独特的刚果红阴性、无分支纤维，直径约 20nm。DnaJ 同源物亚家族 B 成员 9 的免疫组化染色已成为诊断的金标准。存在非典型变异型，包括嗜刚果红、单型和新月形 FGN，突出了该疾病的异质性。免疫抑制治疗，包括利妥昔单抗，已显示出不同的疗效，但尚未建立标准治疗方法。FGN 常导致终末期肾病，诊断后中位进展时间为 2-4 年。FGN 相关的终末期肾病可选择肾移植，但移植肾的复发并不少见。
