Abstract

BackgroundDespite a high prevalence of intracranial aneurysm (ICA) in autosomal dominant polycystic kidney disease (ADPKD), rupture events are rare. The current recommendations for ICA screening are based on expert opinions and studies with low levels of evidence.ObjectivesThe aim of our study was to describe the attitudes of practicing nephrologists in Europe towards screening for ICA using magnetic resonance angiography (MRA).MethodsWe conducted a web-based survey among 1315 European French-speaking nephrologists and nephrology residents. An anonymous, electronic questionnaire including 24 independent questions related to ICA screening modalities, indications and participant profiles was sent by email between September and December 2014. Four hundred and twenty nephrologists (mostly from France) participated, including 31 nephrology residents; the response rate was 32%.ResultsSystematic screening for ICA was advocated by 28% of the nephrologists. A family history of ICA rupture, sudden death, stroke and migraine were consensual indications for screening (> 90% of the panel). In other clinical situations largely not covered by the recommendations (pregnancy, nephrectomy, kidney transplantation, cardiac or hepatic surgery, uncontrolled hypertension, lack of familial ADPKD history, at-risk activity, tobacco use), the attitudes towards screening were highly divergent. ICA screening was influenced by nephrologists experience with ADPKD and by their practice setting. The majority of participants (57%) would not repeat a normal ICA screening. Only a few participants (22%) knew that non-contrast MRA was the reference diagnostic tool for ICA screening, whereas most participants thought that contrast enhancement was necessary to screen for ICA. The results from the nephrology residents were analyzed separately and yielded similar results.ConclusionThis practice survey revealed that most nephrologists follow the current recommendations for the initial screening of ICAs. However, more than a quarter of the panel was in favor of systematic ICA screening, most nephrologists did not know that contrast medium was not necessary to screen for ICA using MRA, and many areas of uncertainty remain.

Highlights

  • Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease

  • Systematic screening for intracranial aneurysm (ICA) was advocated by 28% of the nephrologists

  • ICA screening was influenced by nephrologists experience with ADPKD and by their practice setting

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Summary

Introduction

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. ADPKD frequently leads to end-stage renal failure, accounting for approximately 10% of the renal replacement therapy (RRT) population in Europe [3] and 4.7% of the RRT population in the United States [4]. Several extra-renal manifestations are associated with ADPKD, including polycystic liver disease, mitral valve prolapse, and intracranial aneurysm (ICA) [5]. Intracranial aneurysms are acquired vascular lesions that have a prevalence of approximately 1.8% in the general population [6]. In the ADPKD population, the prevalence of unruptured ICA is between 9% and 12%, i.e., a prevalence ratio of 6.9 compared with the general population [7]. Despite a high prevalence of intracranial aneurysm (ICA) in autosomal dominant polycystic kidney disease (ADPKD), rupture events are rare. The current recommendations for ICA screening are based on expert opinions and studies with low levels of evidence

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