Shah Rafeea, Vydianath Bindu, Pratt Guy, Pinney Jennifer
Department of Renal Medicine, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
Department of Histopathology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
Case Rep Nephrol Dial. 2024 Oct 15;14(1):164-170. doi: 10.1159/000539742. eCollection 2024 Jan-Dec.
Fibrillary glomerulonephritis (FGN) is a rare form of glomerular disease that accounts for less than 1 percent of all renal biopsies. It is characterized by pathognomonic electron microscopy findings of fibrillar deposits in the mesangium and glomerular capillary walls. FGN was initially considered to be an idiopathic disorder. However, approximately 30-50 percent of patients have a secondary cause, including a history of malignancy in up to 23% of cases. Chronic lymphocytic leukemia (CLL) is a rare cause of FGN, with limited data and poor prognosis.
In this report, we present the case of a 69-year-old male who was diagnosed with CLL in 2013 and was initially managed conservatively. In 2016, he developed nephrotic syndrome and renal impairment. Renal biopsy showed FGN, and treatment was targeted to the CLL with bendamustine and rituximab, which led to partial remission of nephrotic syndrome and improvement in renal function. After 3 years of clinical remission, the nephrotic syndrome relapsed, and he underwent a repeat renal biopsy confirming ongoing FGN. A bone marrow biopsy confirmed CLL relapse, and the patient was treated with ibrutinib (a tyrosine kinase inhibitor). The patient achieved a significant organ response and sustained remission.
This case highlights the success of treating a potentially identifiable cause of FGN and highlights that even at relapse, treatment can confer benefits and help prevent end-stage renal failure.
纤维性肾小球肾炎（FGN）是一种罕见的肾小球疾病形式，在所有肾活检病例中占比不到1%。其特征是在系膜和肾小球毛细血管壁出现纤维状沉积物的特征性电子显微镜表现。FGN最初被认为是一种特发性疾病。然而，约30%-50%的患者有继发性病因，其中高达23%的病例有恶性肿瘤病史。慢性淋巴细胞白血病（CLL）是FGN的罕见病因，相关数据有限且预后较差。
在本报告中，我们介绍了一名69岁男性的病例，他于2013年被诊断为CLL，最初接受保守治疗。2016年，他出现肾病综合征和肾功能损害。肾活检显示为FGN，治疗针对CLL，使用苯达莫司汀和利妥昔单抗，这导致肾病综合征部分缓解且肾功能改善。临床缓解3年后，肾病综合征复发，他接受了重复肾活检，证实仍为FGN。骨髓活检证实CLL复发，患者接受了伊布替尼（一种酪氨酸激酶抑制剂）治疗。患者获得了显著的器官反应并持续缓解。
本病例突出了治疗FGN潜在可识别病因的成功，并强调即使在复发时，治疗也能带来益处并有助于预防终末期肾衰竭。
