{"title":"纤维性肾小球肾炎诊断和治疗的最新进展。","authors":"","doi":"10.1053/j.akdh.2024.03.006","DOIUrl":null,"url":null,"abstract":"<div><p>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.</p></div>","PeriodicalId":72096,"journal":{"name":"Advances in kidney disease and health","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Updates on the Diagnosis and Management of Fibrillary Glomerulonephritis\",\"authors\":\"\",\"doi\":\"10.1053/j.akdh.2024.03.006\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><p>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.</p></div>\",\"PeriodicalId\":72096,\"journal\":{\"name\":\"Advances in kidney disease and health\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-07-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Advances in kidney disease and health\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S2949813924000569\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"0\",\"JCRName\":\"UROLOGY & NEPHROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Advances in kidney disease and health","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2949813924000569","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"0","JCRName":"UROLOGY & NEPHROLOGY","Score":null,"Total":0}
引用次数: 0
摘要
纤维性肾小球肾炎(FGN)是一种罕见的肾脏疾病,患者通常为中年人,常表现为晚期肾衰竭、蛋白尿和高血压。纤维性肾小球肾炎可能与自身免疫性疾病、丙型肝炎感染和恶性肿瘤有关。其确切的发病机制仍难以确定,DnaJ 同源物 B 亚家族成员 9 的确切作用也有待确定。肾活检时,FGN 表现出独特的刚果红阴性非分支纤维,直径约为 20 纳米。DnaJ 同源物 B 亚家族成员 9 免疫组化染色已成为诊断的金标准。该病存在非典型变异,包括嗜血型、单型和新月型 FGN,突出了该病的异质性。包括利妥昔单抗在内的免疫抑制治疗效果参差不齐,目前尚未确立标准疗法。FGN 通常会导致终末期肾病,中位进展时间为确诊后 2-4 年。肾移植是治疗与 FGN 相关的终末期肾病的可行方案,但移植肾复发的情况并不罕见。
Updates on the Diagnosis and Management of Fibrillary Glomerulonephritis
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.