Dumas De La Roque C, Brocheriou I, Mirouse A, Cacoub P, Le Joncour A
Département de médecine interne et immunologie clinique, hôpital de la Pitié-Salpêtrière, 75013 Paris, France; Centre de référence maladies auto-immunes systémiques rares, Paris, France; Sorbonne université, Paris, France.
Service d'anatomie pathologique, hôpital de la Pitié-Salpêtrière, 75013 Paris, France; Sorbonne université, Paris, France.
Rev Med Interne. 2024 Nov;45(11):703-709. doi: 10.1016/j.revmed.2024.05.005. Epub 2024 May 15.
Fibrillary glomerulonephritis (FGN) is a glomerular disease described since 1977, with a prevalence in renal biopsies of less than 1%. It presents as renal failure, proteinuria, haematuria and hypertension in middle-aged adults. It is defined histologically, using light microscopy, which reveals organised deposits of fibrils measuring around 20nm, which are negative for Congo red staining. Electron microscopy, the first gold standard for diagnosis, has now been superseded by immunohistochemistry using the anti-DNAJB9 antibody. The discovery of this molecule has revolutionised the diagnosis of GNF, thanks to its excellent sensitivity and specificity (98% and 99% respectively). The association of GNF with hepatitis C virus, autoimmune diseases, neoplasia or haemopathy is debated. Renal prognosis is guarded, with 50% of patients progressing to end-stage renal failure within 2 to 4years of diagnosis. In the absence of randomised controlled trials, the recommended treatment is based on nephroprotective measures, corticosteroid therapy and possibly a second-line immunosuppressant such as rituximab. After renal transplantation, recovery or recurrence is possible. The pathophysiology of the disease is still poorly understood, and further studies are needed.
纤维样肾小球肾炎（FGN）是一种自1977年以来被描述的肾小球疾病，在肾活检中的患病率低于1%。它在中年成年人中表现为肾衰竭、蛋白尿、血尿和高血压。其组织学定义是通过光学显微镜检查，显示出直径约20nm的纤维状有组织沉积物，刚果红染色阴性。电子显微镜曾是诊断的首个金标准，如今已被使用抗DNAJB9抗体的免疫组织化学所取代。该分子的发现彻底改变了FGN的诊断，因其具有出色的敏感性和特异性（分别为98%和99%）。FGN与丙型肝炎病毒、自身免疫性疾病、肿瘤或血液病的关联存在争议。肾脏预后不佳，50%的患者在诊断后2至4年内进展为终末期肾衰竭。在缺乏随机对照试验的情况下，推荐的治疗基于肾脏保护措施、皮质类固醇治疗以及可能使用二线免疫抑制剂如利妥昔单抗。肾移植后，病情有可能恢复或复发。该疾病的病理生理学仍知之甚少，需要进一步研究。
