title: The contribution of arterial calcification to peripheral arterial disease in pseudoxanthoma elasticum.
abstract: OBJECTIVE
  The contribution of arterial calcification (AC) in peripheral arterial disease (PAD) and arterial wall compressibility is a matter of debate. Pseudoxanthoma elasticum (PXE), an inherited metabolic disease due to ABCC6 gene mutations, combines elastic fiber fragmentation and calcification in various soft tissues including the arterial wall. Since AC is associated with PAD, a frequent complication of PXE, we sought to determine the role of AC in PAD and arterial wall compressibility in this group of patients.
  
  
  RESULTS
  Arterial compressibility and patency were determined by ankle-brachial pressure index (ABI) in a cohort of 71 PXE patients (mean age 48 ± SD 14 yrs, 45 women) and compared to 30 controls without PAD. Lower limb arterial calcification (LLAC) was determined by non-contrast enhanced helicoidal CT-scan. A calcification score (Ca-score) was computed for the femoral, popliteal and sub-popliteal artery segments of both legs. Forty patients with PXE had an ABI<0.90 and none had an ABI>1.40. LLAC increased with age, significantly more in PXE subjects than controls. A negative association was found between LLAC and ABI (r = -0.363, p = 0.002). The LLAC was independently associated with PXE and age, and ABI was not linked to cardiovascular risk factors.
  
  
  CONCLUSIONS
  The presence of AC was associated with PAD and PXE without affecting arterial compressibility. PAD in PXE patients is probably due to proximal obstructive lesions developing independently from cardiovascular risk factors.
authors.affiliation: PXE Health and Research Center, University Hospital of Angers, Angers, France; UMR CNRS 6214 - Inserm 1083, School of Medicine, l'UNAM University, Angers, France.
authors.forename: Georges
authors.initials: G
authors.lastname: Leftheriotis
authors.affiliation: UMR CNRS 6214 - Inserm 1083, School of Medicine, l'UNAM University, Angers, France.
authors.forename: Gilles
authors.initials: G
authors.lastname: Kauffenstein
authors.affiliation: Clinical Research Center, University Hospital of Angers, Angers, France.
authors.forename: Jean François
authors.initials: JF
authors.lastname: Hamel
authors.affiliation: PXE Health and Research Center, University Hospital of Angers, Angers, France; UMR CNRS 6214 - Inserm 1083, School of Medicine, l'UNAM University, Angers, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Abraham
authors.affiliation: Department Cell and Molecular Biology, John A. Burns School of Medicine, University of Hawaii, Honolulu, Hawaii, United States of America.
authors.forename: Olivier
authors.initials: O
authors.lastname: Le Saux
authors.affiliation: PXE Health and Research Center, University Hospital of Angers, Angers, France; UPRES 3860, School of Medicine, l'UNAM University, Angers, France.
authors.forename: Serge
authors.initials: S
authors.lastname: Willoteaux
authors.affiliation: UMR CNRS 6214 - Inserm 1083, School of Medicine, l'UNAM University, Angers, France.
authors.forename: Daniel
authors.initials: D
authors.lastname: Henrion
authors.affiliation: PXE Health and Research Center, University Hospital of Angers, Angers, France; UMR CNRS 6214 - Inserm 1083, School of Medicine, l'UNAM University, Angers, France.
authors.forename: Ludovic
authors.initials: L
authors.lastname: Martin
mesh_terms: D000328:Adult; D000368:Aged; D001158:Arteries / Q000473:pathology*; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011561:Pseudoxanthoma Elasticum / Q000473:pathology*; D061205:Vascular Calcification