Zhang Yimeng, Baharani Jyoti, Ajayi Bamidele, Pinney Jennifer
University Hospitals Birmingham NHS Foundation Trust Queen Elizabeth Hospital Birmingham, Mindelsohn Way, Birmingham, B15 2GW, UK.
BMC Nephrol. 2025 Jul 1;26(1):330. doi: 10.1186/s12882-025-04187-z.
Fibrillary glomerulonephritis (FGN) is a rare immune complex-mediated glomerulonephritis characterised by the deposition of anomalous fibrillary structures within the glomeruli. The prognosis for patients with FGN is usually poor with rapid progression to end stage kidney disease (ESKD). There are currently limited data to suggest an optimal therapy strategy to prevent this. Most case series describing FGN come from North America with limited research from the UK.
This is a retrospective case series of patients who presented with biopsy proven FGN to two renal centres within the West Midlands, between 2006 and 2022.
Twenty-one patients with a histological diagnosis of FGN were identified within the 16-year period. Median eGFR at the time of biopsy was 29 mL/min/1.7 (IQR 18-55), serum albumin 31 g/L (IQR 28-33) and ACR was 368 mg/mmol (IQR 303-596). The median follow-up for the cohort was 50 months (range 12-138). DNAJB9 staining was done for five patients, all were positive. Immunosuppression was used in 8 patients following diagnosis of FGN. Treatment varied between steroid, rituximab and cyclophosphamide. Patients with crescents on the biopsy were more likely to receive a trial of immunosuppression. Progression to ESKD was common, 7 (33%) patients required renal replacement therapy within 12 months of diagnosis of FGN.
To date, there are limited numbers of case series of FGN due to the rare nature of the disease. We describe the natural history of this rare kidney condition, and highlight the challenges faced by clinicians where evidence for successful therapeutic options is lacking.
纤维性肾小球肾炎（FGN）是一种罕见的免疫复合物介导的肾小球肾炎，其特征是在肾小球内沉积异常纤维结构。FGN患者的预后通常较差，会迅速进展至终末期肾病（ESKD）。目前，关于预防这种情况的最佳治疗策略的数据有限。大多数描述FGN的病例系列来自北美，英国的研究有限。
这是一项回顾性病例系列研究，研究对象为2006年至2022年间在西米德兰兹郡的两个肾脏中心经活检证实患有FGN的患者。
在这16年期间，共确定了21例经组织学诊断为FGN的患者。活检时的中位估算肾小球滤过率（eGFR）为29 mL/min/1.7（四分位间距18 - 55），血清白蛋白为31 g/L（四分位间距28 - 33），尿白蛋白肌酐比值（ACR）为368 mg/mmol（四分位间距303 - 596）。该队列的中位随访时间为50个月（范围12 - 138个月）。对5例患者进行了DNAJB9染色，结果均为阳性。8例FGN诊断患者在诊断后接受了免疫抑制治疗。治疗方法包括使用类固醇、利妥昔单抗和环磷酰胺。活检时有新月体形成的患者更有可能接受免疫抑制试验。进展至终末期肾病很常见，7例（33%）患者在FGN诊断后12个月内需要肾脏替代治疗。
迄今为止，由于该疾病的罕见性，FGN的病例系列数量有限。我们描述了这种罕见肾脏疾病的自然病程，并强调了临床医生在缺乏成功治疗方案证据时所面临的挑战。
