PowerPoint Slides for: Clinical Features and Outcomes of a Racially Diverse Population with Fibrillary Glomerulonephritis

<b><i>Background:</i></b> Fibrillary glomerulonephritis is characterized by randomly arranged fibrils, approximately 20 nm in diameter by electron microscopy. Patients present with proteinuria, hematuria and kidney insufficiency, and about half of the reported patients progress to end-stage kidney disease within 4 years. The dependence of patient characteristics and outcomes on race has not been explored. In this study, we describe a cohort of patients with fibrillary glomerulonephritis and compare their clinical characteristics and outcomes with those of patients previously described. <b><i>Methods:</i></b> The University of North Carolina (UNC) Nephropathology Database was used to retrospectively identify patients diagnosed with fibrillary glomerulonephritis between 1985 and 2015. Of these patients, those treated at UNC were selected. Their demographic and clinical characteristics - including signs and symptoms, comorbidities, laboratory values, treatments and outcomes - were compared with those of patients described earlier. <b><i>Results:</i></b> Among the 287 patients identified, 42 were treated at the UNC Kidney Center. When compared to earlier cohorts, a higher frequency of black race, hepatitis C virus (HCV) infection and use of hemodialysis were noted in both black and HCV-positive patients. Autoimmune diseases, infections and malignancies were frequently observed, present in over half of all cases. <b><i>Conclusion:</i></b> According to this study, fibrillary glomerulonephritis represents a secondary glomerular disease process (associated with autoimmune disease, infection or malignancy) in many cases and hence screening is essential. As the screening for comorbidities increased over time, more underlying causes were identified. We noted a high frequency of HCV among black patients, suggesting a possible causative association. Treatment of underlying disease is essential for patients for the best outcome.

背景：纤维样肾小球肾炎（fibrillary glomerulonephritis）的特征为电子显微镜下可见直径约20 nm的随机排列纤维丝。患者临床表现为蛋白尿、血尿与肾功能不全，约半数已报道的患者会在4年内进展为终末期肾病。目前尚未有研究探讨患者特征与临床转归是否存在种族相关性。本研究描述了一组纤维样肾小球肾炎患者队列，并将其临床特征与转归与既往报道的患者进行对比。
方法：本研究使用北卡罗来纳大学（University of North Carolina, UNC）肾病理数据库，回顾性筛选1985年至2015年间确诊为纤维样肾小球肾炎的患者，其中选取在UNC接受治疗的病例。对其人口统计学特征与临床特征——包括体征与症状、合并症、实验室检查指标、治疗方案及临床转归——与既往报道的患者队列进行对比分析。
结果：本研究共筛选出287例患者，其中42例在UNC肾脏中心接受治疗。与既往队列相比，本队列中黑人种族、丙型肝炎病毒（hepatitis C virus, HCV）感染率以及血液透析使用率均更高，且这一差异在黑人患者与HCV阳性患者中均有体现。自身免疫性疾病、感染与恶性肿瘤较为常见，半数以上病例存在此类基础疾病。
结论：本研究结果显示，多数病例中的纤维样肾小球肾炎属于继发性肾小球疾病（与自身免疫性疾病、感染或恶性肿瘤相关），因此针对潜在病因的筛查至关重要。随着合并症筛查手段的不断完善，越来越多潜在致病因素得以明确。本研究观察到黑人患者中HCV感染率较高，提示二者可能存在因果关联。对于患者而言，积极治疗基础疾病是获得最佳临床转归的核心措施。