Impact of Kidney Function and Kidney Volume on Intracranial Aneurysms in Patients with Autosomal Dominant Polycystic Kidney Disease

Abstract Background To date, only a personal or family history of intracranial aneurysm/subarachnoid hemorrhage (IA/SAH) has been established as a risk factor for IA in autosomal dominant polycystic kidney disease (ADPKD). Recently, an association between splicing/frameshift mutations, which are reportedly associated with declining kidney function/increased total kidney volume (TKV), and IAs in patients with ADPKD was reported. Objective To confirm the association between kidney function/volume and IAs in patients with ADPKD. Methods This study included 519 patients with ADPKD. Kidney function, TKV, and general risk factors for IA were assessed for associations with IA formation. Results At baseline IA screening, the median age, estimated glomerular filtration rate, and TKV were 44 years, 54.5 mL/min/1.73 m2, and 1054.3 mL, respectively. A family IA/SAH history was confirmed in 18.1% patients, and 54.3% patients had hypertension. IA point prevalence was 12.5%. During a clinical follow-up of 3104 patient-years, de novo IA was detected in 29 patients (0.93% patient-years). IA period prevalence was 18.1% (median age, 60 years). Multivariable logistic regression analyses demonstrated that TKV ≥ 1000 mL (odds ratio [OR] = 2.81), height-adjusted TKV ≥ 500 mL (OR = 2.81), Mayo 1D–1E (OR = 2.52), and chronic kidney disease stages 3–5 (OR = 2.31) were significantly associated with IA formation. Conclusions IAs in patients with ADPKD may be associated with risk factors for IAs in the general population as well as declining kidney function and increased kidney volume. Kidney disease progression may contribute to effective IA screening and treatment planning in patients with ADPKD.