KDIGO 2025 Clinical Practice Guideline for ADPKD: a commentary on intracranial aneurysms and other vascular manifestations from the ERA Working Group Genes & Kidney

The 2025 KDIGO Clinical Practice Guideline for Autosomal Dominant Polycystic Kidney Disease (ADPKD) addresses intracranial aneurysms (ICAs) and other vascular manifestations, an aspect of ADPKD care which is of considerable clinical importance and associated with significant patient anxiety. This commentary from the ERA Working Group Genes&Kidney provides a European perspective on guideline implementation, highlighting key areas requiring action and research. People with ADPKD face a 4-5-fold increased risk of ICA (prevalence ∼12.9%) compared to the general population, yet absolute rupture rates remain low (0.57 per 1,000 person-years). The guidelines appropriately emphasize risk-stratified, individualized approaches centered on family history, smoking history, and blood pressure control. Key points include importance of patient information; selective screening in those with family history of ICA, subarachnoid hemorrhage, or unexplained sudden death; and use of contrast-free MR angiography as the preferred imaging modality. Rescreening intervals of 5-10 years are suggested for high-risk individuals with negative initial screening. Treatment decisions require multidisciplinary expertise at high-volume centers, balancing rupture risk against intervention complications. Thoracic aortic aneurysms show clear association with ADPKD, warranting family screening when present, while abdominal aortic aneurysms demonstrate no increased prevalence. Patient and clinician education emerges as paramount, moving beyond anxiety-driven approaches toward informed shared decision-making. Future research priorities include leveraging European registries to identify genetic risk modifiers, developing validated risk prediction tools, and establishing whether screening translates to improved clinical outcomes in ADPKD-specific populations.