Association of Autosomal Dominant Polycystic Kidney Disease With Incident Aortic Dissection or Aneurysm

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by a broad range of extrarenal complications, yet epidemiologic data on its association with aortic dissection (AD) or aortic aneurysm (AA) remain limited. In the present study, we assessed whether ADPKD is associated with an increased risk of developing these aortic conditions.

Observational cohort study.

2,568,283 individuals without a history of AD or AA, enrolled between April 2014 and August 2023 in a nationwide Japanese epidemiological database provided by DeSC Healthcare of Tokyo, Japan.

Presence of ADPKD based on the International Classification of Diseases, Tenth Revision codes.

Incidence of AD or AA.

Cause-specific hazards models were used to estimate associations (hazard ratios), adjusting for potential confounders.

The median age was 68 years (IQR, 61-77), and 1,123,131 individuals (44%) were male. ADPKD was diagnosed in 1,102 individuals (0.04%) within the cohort. During a median follow-up of 1,043 days (IQR: 556-1,600), there were 15,019 occurrences of AD or AA. Multivariable cause-specific hazards models demonstrated that individuals with ADPKD had a significantly increased risk of developing these aortic diseases (HR, 1.76 [95% CI, 1.13-2.73]). When analyzed separately, the HRs were 2.53 (95% CI, 1.13-5.66) for AD and 1.56 (95% CI, 0.94-2.60) for AA. The association of ADPKD with incident AD or AA was more pronounced in individuals with body mass index (BMI) ≥ 25 kg/m² than in those with BMI < 25 kg/m².

Potential residual confounding, possible detection bias, and lack of data on ADPKD-causing genetic variant.

Our analysis of a large-scale epidemiological dataset indicated an elevated risk of AD or AA occurrence in individuals with ADPKD. These findings may inform the clinical management of this condition.

Autosomal dominant polycystic kidney disease (ADPKD) is a common inherited condition that can impair kidney function and affect other organs. Previous studies have suggested that aortic dissection (AD) or aortic aneurysm (AA) may be among its complications, but the evidence has remained inconclusive. We used a nationwide dataset of more than 2.5 million individuals to examine whether individuals with ADPKD are at increased risk of developing AD or AA. Our analyses showed a significant association between ADPKD and these serious aortic disorders. These findings highlight the importance of multidisciplinary efforts to promote prevention, timely recognition, and early intervention for AD or AA in individuals with ADPKD.