Incidence, prevalence and mortality of chronic periaortitis: a population-based study

OBJECTIVES: To evaluate the epidemiology, presentation and outcomes of patients with chronic periaortitis from 1998 through 2018. METHODS: An inception cohort of patients with incident chronic periaortitis from January 1, 1998 through December 31, 2018, in Olmsted County, Minnesota was identified based on comprehensive individual medical record review utilising the Rochester Epidemiology Project medical record linkage system. Inclusion required radiographic and/or histologic confirmation of periarterial soft tissue thickening around at least part of the infra-renal abdominal aorta or the common iliac arteries. Data were collected on demographic characteristics, clinical presentation, renal and radiographic outcomes, and mortality. Incidence rates were age and sex adjusted to the 2010 United States white population. RESULTS: Eleven incident cases of chronic periaortitis were identified during the study period. Average age at diagnosis was 61.8±13.4 years. The cohort included 9 men (82%) and 2 women (18%). Age- and sex-adjusted incidence rates per 100,000 population were 0.26 for females, 1.56 for males and 0.87 overall. Overall prevalence on January 1, 2015 was 8.98 per 100,000 population. Median (IQR) length of follow-up was 10.1 (2.5, 13.8) years. Overall mortality was similar to the expected age, sex, and calendar estimates of the Minnesota population with standardised mortality ratio (95% CI) for the entire cohort 2.07 (0.67, 4.84). CONCLUSIONS: This study reports the first epidemiologic data on chronic periaortitis in the United States. In this cohort of patients with chronic periaortitis, men were 4 times more commonly affected than women. Mortality was not increased compared to the general population.