A trend in progressive decrease in mortality rates associated with primary systemic vasculitis, as well as sex, racial, and geographic disparities, was observed in the United States, according to results of an analysis published in the Journal of Clinical Medicine.

Researchers sought to estimate the mortality rates of primary systemic vasculitis and its geographic distribution, using recent US population data.

An epidemiologic study was conducted using the Centers for Disease Control and Prevention (CDC) Wonder Multiple Cause of Death (MCD) database and its query system to obtain mortality rates for vasculitis from 1999 to 2019. Mortality data were derived from death certificates that were filed in the US and the District of Columbia between 1999 and 2019. The CDC MCD data included country-level national mortality and population data. Each death certificate identified a single underlying cause of death (UCD). According to the World Health Organization, a UCD is defined as “the disease or injury which initiated the train of events leading directly to death, or the circumstances of the accident or violence which produced the fatal injury.”

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The 21-year study period was based on a cumulative population of 6,416,872,524 individuals, among whom vasculitis was reported as the CDC MCD in a total of 28,190. Of these patients, vasculitis was reported as the UCD among 13,048 (46.29%).

Results of the analysis reported age-adjusted mortality rate of vasculitis as the MCD in 4.077 per million persons (95% CI, 4.029-4.125 per million persons) and as the UCD in 1.888 per million persons (95% CI, 1.855-1.921 per million persons). A significant declining trend was reported for vasculitis both as an MCD, with an average annual percent change (AAPC) of -3.7% (95% CI, -4.5% to -2.8%), and as a UCD, with an AAPC of -3.8% (95% CI, -5% to -2.5%).

Further, age-adjusted mortality rate was higher among women than among men regarding vasculitis as the MCD (5.057 vs 3.641 per million persons, respectively) and the UCD (2.220 vs 1.841 per million persons, respectively). The percentage of deaths from vasculitis was also higher among women than among men for vasculitis as the MCD (58.95% vs 41.05%, respectively) and the UCD (55.50% vs 44.51%, respectively).

Regarding racial differences, the most deaths occurred among White patients due to vasculitis as the MCD (92.34%). The highest mortality rate was also reported among White patients (4.371; 95% CI, 4.317-4.424) for the MCD, as well as for the UCD (91.36%). However, the mortality rate, was slightly higher among patients who were American Indian or Alaskan Native (2.059; 95% CI, 1.643-2.475) compared with those who were White (2.032; 95% CI, 1.995-2.069). Higher mortality rates were observed in northern US states and in areas with lower populations

A major limitation of the current study included the fact that data on the overall prevalence and type of vasculitis among the groups, and the geographic regions evaluated, were not available.

Researchers concluded, “Further analyses are warranted to better understand the factors associated with these disparities in order to implement targeted public health interventions to decrease them.”


Rodriguez-Pla A, Rossello-Urgell J. Trend and geographic disparities in the mortality rates of primary systemic vasculitis in the United States from 1999 to 2019: a population-based study. J Clin Med. 2021;10(8):1759. doi:10.3390/jcm10081759