Background/Purpose: Chronic periaortitis is an inflammatory condition that typically involves the infrarenal portion of the abdominal aorta. To date, no epidemiologic studies have been performed in North America. The purpose of this study was 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, MN, USA, was identified based on comprehensive individual medical record review utilizing 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%). The most common presenting symptom was pain with 55% (6/11) reporting abdominopelvic-pelvic pain, 36% (4/11) back pain, and 18% (2/11) flank pain. Obstructive uropathy was present in 73% (8/11) subjects: 3 (27%) unilateral left, 1 (9%) unilateral right, and 4 (36%) bilateral. Mean±SD creatinine at presentation was 2.7±3.4 mg/dL. Ureteral stenting was required at diagnosis in seven patients, unilateral left in 2, unilateral right in 1 and bilateral in 4. All 11 patients received glucocorticoids with a median (IQR) dose of 40 (30, 60) mg/day. Additional non-glucocorticoid therapeutics were used in 10 patients.

Renal function stage at last follow up declined in 2 patients, remained the same in 3 patients and improved in 6 patients. Mean (±SD) creatinine at last follow-up was 1.2±0.2 mg/dL. Among the 7 patients requiring baseline indwelling ureteral stent placement only two required ongoing ureteral stenting at last follow up. None of the four patients without ureteral stenting at diagnosis progressed to require stenting during the follow-up period. No patient underwent ureterolysis surgery in this cohort. Periarterial soft tissue thickening at last follow up had increased in thickness in 1 (9%), was unchanged in 2 (18%), decreased in size but did not resolve in 6 (55%), and fully resolved in 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 standardized mortality ratio (95% CI) for the entire cohort 2.07 (0.67, 4.84).

Conclusion: 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.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/incidence-prevalence-and-mortality-of-chronic-periaortitis-a-population-based-study/