Racial and ethnic disparities in life expectancy in the U.S. have been widely documented. To date, there remains a paucity of similar data in patients with inborn errors of immunity (IEI).
To examine racial and ethnic differences in IEI mortality in the U.S.
We analyzed the National Center for Health Statistics national mortality data from 2013 to 2018. We quantified age-adjusted death rate (ADR) and age-specific death rate (ASDR) from IEI for each major racial and ethnic group in the U.S, and examined the association of race and ethnicity with death at a younger age. For detailed Methods, please see the Methods section in this article's Online Repository at www.jacionline.org.
During 2003 to 2018, IEI was reported as the underlying or contributing cause of death in 14,970 individuals nationwide. ADR was highest among Black patients (4.25 per 1,000,000 person-years), compared with 2.01, 1.71, 1.50, and 0.92 per 1,000,000 person-years for White, American Indian/Alaska Native (AIAN), Hispanic, and Asian/Pacific Islander (API) patients, respectively. Odds of death before age 65 years was greatest among Black patients (OR 5.15; 95% CI 4.61-5.76), followed by AIAN patients (OR 3.58; 95% CI 2.30-5.82), compared with White patients. Odds of death before age 24 years was greatest among Hispanic patients, compared with non-Hispanic patients (OR 3.60; 95% CI 3.08-4.18).
Our study highlights racial and ethnic disparities in IEI mortality and the urgent need to further identify and systematically remove barriers in care for historically marginalized patients with IEI.