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. 2024 Dec 12;20(2):229-238.
doi: 10.2215/CJN.0000000590. Online ahead of print.

Incidence and Proportion of Primary Focal Segmental Glomerulosclerosis (FSGS) among a Racially and Ethnically Diverse Adult Patient Population between 2010 and 2021

Mercedes A Munis  1 , Qiaoling Chen  1 , T Matthew Hill  2 , Min Zhuo  3 , Asher D Schachter  3 , Simran K Bhandari  4 , Aviv Hever  5 , Teresa N Harrison  1 , Ancilla W Fernandes  2 , John J Sim  1   6   7
Affiliations

Incidence and Proportion of Primary Focal Segmental Glomerulosclerosis (FSGS) among a Racially and Ethnically Diverse Adult Patient Population between 2010 and 2021

Mercedes A Munis et al. Clin J Am Soc Nephrol. .

Abstract

Key Points:

  1. Primary FSGS is a rare immune mediated glomerulopathy that accounted for 16.6% for all FSGS reported biopsies among a diverse patient population.

  2. From 2010 to 2021, the standardized incidence of primary FSGS was estimated at 1.7 cases per 100,000 patient-years.

  3. The highest incidence of 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.

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Conflict of interest statement

Disclosure forms, as provided by each author, are available with the online version of the article at http://links.lww.com/CJN/C61.

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