Risk Factors ID’d for Early-Onset Intracranial Aneurysm in ADPKD – Renal and Urology News

Advanced chronic kidney disease (CKD) and family history are risk factors for early-onset intracranial aneurysm in younger patients with autosomal dominant polycystic kidney disease (ADPKD), a new study finds.

In the study of 519 patients with ADPKD, 94 had an intracranial aneurysm. The odds of early-onset intracranial aneurysm were significantly increased 3.9-fold with stage 4-5 CKD and 2.3-fold with a family history of intracranial aneurysm or subarachnoid hemorrhage among patients younger than 50 years, Hiroshi Kataoka, MD, of Tokyo Women’s Medical University in Japan and colleagues reported in the Journal of Nephrology.¹ They noted that smoking is an established risk factor for early-onset intracranial aneurysm in the general population.

For patients aged 50 years or older, the odds of intracranial aneurysm were significantly increased 2.4- and 3.5-fold with stage 4-5 CKD and family history of intracranial aneurysm or subarachnoid hemorrhage, respectively. In addition, female sex and hypertension were significantly associated with 4.5- and 5.9-fold increased odds of intracranial aneurysm, respectively.

According to Dr Kataoka’s team, “a family history of intracranial aneurysm or subarachnoid hemorrhage predisposes an individual to early-onset intracranial aneurysms, multiple aneurysms, increased risk of rupture in identified aneurysms, and poorer outcomes after rupture.”

“Considering that advanced kidney dysfunction in younger patients is generally associated with truncating PKD1 mutations including splicing/frameshift mutations, further analyses are needed to disentangle whether the genotype or the kidney dysfunction is associated with intracranial aneurysms in patients with ADPKD,” they wrote.

Younger patients with these risk factors should be considered for extensive intracranial aneurysm screening, according to Dr Kataoka’s team.

In Advances in Kidney Disease and Health,² Terry Watnick, MD, of the University of Maryland School of Medicine in Baltimore, and colleagues discussed screening patients with a positive family history with a 3D time-of-flight magnetic resonance angiography with a 3T system without gadolinium administration. They noted that the risk of aneurysm is 16%-23% with a family history and 5-6% without.

If an intracranial aneurysm is found, the patient should receive multidisciplinary care involving a nephrologist, neurosurgeon, and interventional neuroradiologist, according to the authors. A severe “thunderclap” headache or new onset of neurologic symptoms should prompt immediate evaluation.

In Kidney360, separate teams of clinicians debated the pros and cons of universal screening of the ADPKD population.^3-4 Advocates Meghana Eswarappa, MD, and Meyeon Park, MD, of the University of California, San Francisco, wrote that “considering the potential for morbidity and mortality in this unique population at risk for [intracranial aneurisms] with possibly higher-than-average risk of rupture, we believe that presymptomatic screening for [intracranial aneurisms] in all individuals with ADPKD regardless of family history is warranted.” The frequency of follow-up screening in cases of incidental small intracranial aneurysms or to detect de novo intracranial aneurysms “is likely justifiable at a 5-year interval based on cost-effectiveness analyses.”

Fouad T. Chebib, MD, and Rabih G. Tawk, MD, of Mayo Clinic in Jacksonville, Florida, offered an opposing view. “The variable prevalence rates of [intracranial aneurysms], their rate of rupture, the potential physical and psychological risks associated with screening and subsequent interventions, the economic implications, and the nuances of local medical expertise make a universal approach potentially imprudent,” they wrote. “The potential harms, in certain situations, could outweigh the benefits.”

Disclosure: Some study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures.