gms | German Medical Science

Objective: Autosomal polycystic kidney disease (APKD) is a hereditary condition leading to vascular abnormalities. APKD patients are known to have more intracranial aneurysms (IA). The Kuopio cohort (Finland) is nowadays the largest cohort having compared demographic data and aneurysms characteristics between APKD- and non-APKD patients [1]. Characteristics of APKD patients has been reviewed after a systematic review of the literature in 2017 [2]. The @neurIST Aneurysm Data Bank (ADB) aims to collect information of patients diagnosed with IA. This study compares observation made in the ADB cohort, the Kuopio cohort and the literature review to have a better understanding of IA in APKD patients.

Methods: Information collected in the ADB on prospectively and consecutively recruited patients with ruptured and unruptured IA was studied. APKD patients were identified from it. Demographic data and aneurysm characteristics were analyzed and compared to the Kuopio cohort, which enrolled 6286 non-APKD IA and 102 APKD IA and the systematic review that reported information on 563 APKD patients, 679 IAs and location of 345 IAs.

Results: Between 2007 and 2017, 968 patients with IA were enrolled in the prospective and consecutive dataset of the ADB (947 non-APKD, 21 PKD) for a total of 1229 IA (1191 non-APKD and 38 APKD). PDK patients had significantly more IA in MCA arteries (OR 2.0, 95%CI 1.0-3.8) than other locations. Despite differences in the distribution of aneurysm according to location in different populations, the predominance of IA located in MCA arteries has been observed consistently. In all cohorts, APKD patients had more often multiple IA (p<0.05) and were younger than non-APDK. APKD patients suffered more often from hypertension than non-APKD (Kupio: 73.6% vs. 34.0%; ADB: 61.9% vs. 39.3%). No statistical difference was found in term of size of IA.

Conclusion: Patients suffering of APKD are more likely to suffer intracranial aneurysms than non-affected subjects. APKD patients diagnosed with IA are more likely to have multiple IAs and IAs are more likely to be located in MCA arteries. The precise role of loss of primary cilia function and blood hypertension on disease initiation, progression and severity still needs further investigations.