f primary FSGS was observed among Black (3.2) and Asian (2.7 cases per 100,000 patient-years) people. Background Focal segmental glomerulosclerosis (FSGS) refers to a pattern of glomerular injury but also includes primary FSGS which is considered as an immune-mediated glomerulopathy. We sought to determine the incidence of primary FSGS and proportion of patients with FSGS who have primary FSGS among a large diverse patient population in the United States. Methods A cross-sectional study (2010–2021) was performed within an integrated health system in patients (age 18 or older) with biopsy-proven FSGS. Among biopsies with FSGS as the first diagnosis on pathology report, chart reviews were performed to determine primary FSGS, defined as podocyte foot process effacement ≥80% on electron microscopy. The proportion of patients with primary FSGS and annual incidence rate (IR) (per 100,000 patient-years) were calculated. Standardized IR were determined by age, sex, and race and ethnicity based on US population structure of the 5-year (2018–2022) American Community Survey estimates. Results We identified 3838 patients with FSGS reported on biopsy. Among 1502 with FSGS as the principal diagnosis, 637 met criteria for primary FSGS (mean [SD] age 55.5 years [17.9], 56.5% male, 35.6% Hispanic, 28.7% White, 17.9% Asian/Pacific Islander, and 16.0% Black). The mean standardized IR (confidence interval) of primary FSGS was 1.7 (0.9 to 2.5) per 100,000 patient-years during the study period. The standardized annual IR ranged from 1.3 to 2.4 per 100,000 patient-years. IR (per 100,000 patient-years) were highest among Black (3.2), Asian (2.7), and Pacific Islander (2.8) patients. Conclusions Primary FSGS accounted for 16.6% of biopsy-proven FSGS. Primary FSGS is a likely a rare disease with incidence highest among Black, Asian, and Pacific Islander people. More precise identification of primary FSGS may facilitate work to improve understanding of this glomerulopathy and improve kidney outcomes....

中文翻译：

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2010 年至 2021 年种族和民族多元化成年患者群体中原发性局灶节段性肾小球硬化 （FSGS） 的发病率和比例


f 在黑人 （3.2） 和亚洲 （2.7/100,000 患者年） 人群中观察到原发性 FSGS。背景：局灶节段性肾小球硬化 （FSGS） 是指肾小球损伤的一种模式，但也包括原发性 FSGS，它被认为是一种免疫介导的肾小球病。我们试图确定原发性 FSGS 的发病率以及在美国大量不同患者群体中患有原发性 FSGS 的 FSGS 患者的比例。方法 在综合卫生系统内对经活检证实的 FSGS 患者（18 岁或以上）进行了一项横断面研究 （2010-2021）。在以 FSGS 作为病理报告首诊诊断的活检中，进行图表审查以确定原发性 FSGS，定义为足细胞足突消失 ≥80% 在电子显微镜下。计算原发性 FSGS 患者的比例和年发病率 （IR） （每 100,000 患者年）。标准化 IR 是根据 5 年（2018-2022 年）美国社区调查估计的美国人口结构按年龄、性别、种族和民族确定的。结果 我们确定了 3838 例活检报告的 FSGS 患者。在以 FSGS 为主要诊断的 1502 例患者中，637 例符合原发性 FSGS 的标准（平均 [SD] 年龄 55.5 岁 [17.9]，56.5% 男性，35.6% 西班牙裔，28.7% 白人，17.9% 亚洲/太平洋岛民和 16.0% 黑人）。在研究期间，原发性 FSGS 的平均标准化 IR （置信区间） 为 1.7 （0.9 至 2.5） 每 100,000 名患者年。标准化的年度 IR 范围为每 100,000 患者年 1.3 至 2.4 次。IR （每 100,000 患者年） 在黑人 （3.2） 、亚裔 （2.7） 和太平洋岛民 （2.8） 患者中最高。结论 原发性 FSGS 占活检证实的 FSGS 的 16.6%。 原发性 FSGS 可能是一种罕见疾病，在黑人、亚洲和太平洋岛民中发病率最高。更精确地识别原发性 FSGS 可能有助于提高对这种肾小球病的理解并改善肾脏结局的工作。