Characteristics and Distribution of Intracranial Aneurysms in Patients with Autosomal Dominant Polycystic Kidney Disease Compared with the General Population: A Meta-Analysis.

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic condition associated with intracranial aneurysms (IAs). The associated pathophysiology remains unknown but an association with wall shear stress (WSS) is suspected. Cerebral arterial location is the principal factor influencing IA natural history. This study aims to compare IA location specific distribution between ADPKD- and non-ADPKD-patients. The ADPKD-group was composed of data from a systematic review of the literature (2016-2020, N=7), and three cohorts: @neurIST, Novosibirsk and UCAS. The non-ADPKD-group was formed from the @neurIST, UCAS, ISGC and the Finnish cohort from the literature. Patients and IAs characteristics were compared between ADPKD- and non-ADPKD-groups, and a meta-analysis for IA locations was performed. 1184 IAs from ADPKD-patients were compared to 21040 IAs from non-ADPKD-patients. 78.6% of ADPKD-patients had hypertension versus 39.2% of non-ADPKD-patients. 32.4% of ADPKD-patients were smokers versus 31.5% of non-ADPKD-patients. 30.1% of ADPKD-patients had a positive family history for IA versus 15.8% of the non-ADPKD-patients. ADPKD-patients showed a higher rate of IA multiplicity (33.2% versus 23.1%). IAs from ADPKD-patients showed a significant predominance across the internal carotid (ICA) and middle cerebral (MCA) arteries. Posterior communicating (Pcom) IAs were more frequently found in the non-ADPKD-group. The meta-analysis confirmed a predominance of IAs in the ADPKD-patients across large caliber arteries (OR (95% CI): ICA: 1.90 (1.10-3.29); MCA: 1.18 (1.02-1.36)). Small diameter arteries such as the Pcom were observed more in non-ADPKD-patients (0.21 (0.11-0.88)). This analysis shows that IAs diagnosed in ADPKD-patients are more often localized in large caliber arteries from the anterior circulation in comparison to IAs in non-ADPKD-patients. It shows that primary cilia driven wall shear stress vessel remodeling to be more critical in cerebral anterior circulation large caliber arteries.