Although patients with autosomal dominant polycystic kidney disease (ADPKD) have a higher incidence of intracranial aneurysms than found in the healthy population, research concerning the factors related to the risk of intracranial rupture in patients with ADPKC is still insufficient.

In a paper published in Cerebrovascular Diseases, the researchers aimed to investigate the prevalence of intracranial aneurysms and aneurysmal subarachnoid hemorrhage (SAH) and analyze the systemic factors associated with high-risk aneurysms in patients with ADPKD. “Previous studies have rarely attempted to identify the factors related to the presence of aneurysms and aneurysmal SAH in patients with ADPKD,” they write. “We sought to identify ADPKD-related system markers associated with the development and high-risk phenotype of intracranial aneurysms.”

Patients Divided into Three Risk Categories

The researchers screened patients with ADPKD who visited a single tertiary hospital and who underwent cerebral angiography between January 2007 and May 2017.  The patient population included those aged 15 to 19 years with three or more cysts found on one or both sides of the renal parenchyma, patients aged 49 to 59 years with two or more cysts found in each kidney, and patients aged 60 years or older with four or more cysts in each kidney. Patients were examined for the presence of intracranial aneurysms and subsequently reclassified into three groups based on the risk of aneurysm rupture: 1) aneurysm-negative, 2) low-risk aneurysm, and 3) high-risk aneurysm. Various systemic factors were compared, and independent factors linked with high-risk aneurysm were analyzed.

“Among 926 patients with ADPKD included in the final analysis, the prevalence of intracranial aneurysms and aneurysmal SAH was 16% and 1.2%, respectively,” the study authors write. “Age, female sex, dolichoectasia, and mitral valve deceleration time were positively associated with high-risk aneurysms, whereas hypercholesterolemia was negatively associated.”

Early Diagnosis Prior to Rupture Is Essential

Although the prevalence of intracranial aneurysms in patients with ADPKD varies, it is approximately three to four times greater than in the healthy population, while the incidence of aneurysmal rupture in these patients is greater than five times that of healthy controls, according to the study authors. “Aneurysmal rupture accounts for 85% of SAH and half of the patients with untreated SAH die within a month,” they write. “Due to the high incidence and probability of aneurysmal rupture on ADPKD, early diagnosis prior to rupture is an essential aspect of patient care. Therefore, metabolic parameters have to be carefully examined and considered for the suitable care of patients with ADPKD.”

The study authors would like to see future studies address the factors involved in the prevalence of intracranial rupture due to aneurysm in patients with ADPKD. “A longitudinal prospective study is needed to define whether intracranial aneurysms evolve into high-risk aneurysms or aneurysmal SAH,” they write. “In addition, as this was a single-center study, we would welcome a multicenter investigation conducted in order to enhance the applicability of the findings to a larger population.”



Clinical Factors Associated With the Risk of Intracranial Aneurysm Rupture in Autosomal Dominant Polycystic Kidney Disease