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Updated: Jun 6, 2025

Calcification of Vascular Smooth Muscle Cells and Imaging of Aortic Calcification and Inflammation
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Arterial calcification volume is associated with a higher risk of cardiovascular events in pseudoxanthoma elasticum

Iris M Harmsen1, Frank L J Visseren1, Madeleine Kok2

  • 1Department of Vascular Medicine, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands.

Atherosclerosis
|November 24, 2024
PubMed

Insights

In pseudoxanthoma elasticum (PXE), higher arterial calcification on CT scans predicts future cardiovascular events in patients without prior cardiovascular history. This finding supports using arterial calcification as an endpoint in clinical trials.

Area of Science:

  • Vascular Medicine
  • Genetics
  • Radiology

Background:

  • Pseudoxanthoma elasticum (PXE) is linked to arterial calcification due to ABCC6 gene mutations, but its association with vascular complications is unclear.
  • PXE disease progression necessitates reliable intermediate endpoints for clinical trials.
  • Arterial calcification on computed tomography (CT) is a potential surrogate marker for cardiovascular events in PXE.

Purpose of the Study:

  • To determine the relationship between arterial calcification measured by CT and future cardiovascular events in PXE patients.

Main Methods:

  • A prospective cohort study included 326 PXE patients from the UMC Utrecht Expertise Center for PXE (UECP).
  • Arterial calcification volume was quantified using low-dose full-body CT scans.
  • Cox proportional hazard models analyzed the association between arterial calcification and incident cardiovascular events during a median follow-up of 6.0 years.

Main Results:

  • 41 cardiovascular events occurred (21 per 1000 person-years).
  • In PXE patients with no prior cardiovascular history, increased arterial calcification volume at baseline significantly correlated with future cardiovascular events (adjusted HR: 1.87).
  • No significant association was found between arterial calcification and future events in patients with pre-existing cardiovascular disease.

Conclusions:

  • Elevated arterial calcification on CT scans in PXE patients indicates an increased risk of a first cardiovascular event.
  • Arterial calcification shows promise as an intermediate endpoint for evaluating interventions aimed at reducing cardiovascular risk in PXE clinical studies.
Abstract

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