Demographic Disparities in the Incidence of Moyamoya Angiopathy in the United States.

BACKGROUND AND

OBJECTIVES

There is paucity of data on the population-level incidence of moyamoya angiopathy (MMA) in the United States. The aim of this study was to estimate sex-specific, age-specific, and race-specific incidence of MMA in the United States over the period 2011-2020.

METHODS

We used the State Ambulatory Surgery, Emergency, and Inpatient Databases of Florida (2005-2020), Georgia (2010-2020), Maryland (2012-2020), and New York (2005-2020) to conduct a retrospective study. We validated ICD-10 codes for MMA in a single academic center in the United States and used primary and secondary ICD-9/10 discharge codes to identify all new cases of MMA in these 4 states from 2011 to 2020. Incident MMA counts were combined with census data to compute incidence separately for moyamoya disease (MMD) and moyamoya syndrome (MMS). Multivariable-adjusted Poisson regression models were used to compare MMA incidence between demographic subgroups, and joinpoint regression was used to evaluate trends over time.

RESULTS

Among 24,614 MMA hospitalizations over the study period, 4,192 incident cases were identified from 2011 to 2020. The median age at hospitalization was 41.0 years (interquartile range 24.0-54.0), and 67.9% of admissions were in women. The overall age-standardized and sex-standardized incidence of MMA was 0.75 (95% CI 0.73-0.77) cases/100,000/year. The incidence of isolated MMD was 0.57 (95% CI 0.54-0.59), whereas that of MMS was 0.18 (95% CI 0.16-0.20). MMD incidence increased with age, up to 45-64 years (trend p value = 0.041), whereas that of MMS declined with age (trend p value <0.001). Following multivariable Poisson regression, the overall MMA incidence in women was approximately 2 times that in men (adjusted incidence rate ratio [aIRR] 1.97, 95% CI 1.69-2.30). Incidence was higher in Asian (aIRR 2.46, 95% CI 1.88-3.23) and Black (aIRR 2.58, 95% CI 2.17-3.07) individuals compared with non-Hispanic White individuals. Incidence increased over time (annualized percentage change 7.7%, 95% CI 5.3%-10.5%), with most of this increase occurring in individuals aged 45-64 years and 65 years or older.

DISCUSSION: The incidence of MMA in the United States is higher than previously believed. Overall incidence in Black individuals is just as high as that in Asian individuals, and both are higher than that of non-Hispanic White individuals. Further studies are needed to understand the genetic and environmental factors contributing to race/ethnic disparities in MMA incidence in the United States.