title: Increased carotid intima-media thickness in the absence of atherosclerotic plaques in an adult population with Fabry disease.
abstract: OBJECTIVE
  Fabry disease is considered primarily as a progressive small vessel disease, with ischaemic degenerative lesions involving the kidneys, brain and heart. Macrovascular involvement in male patients includes an accelerated wall hypertrophy of the radial artery and a thickening of the intima-media of the common carotid artery. The aim of this study is to evaluate the prevalence and severity of carotid artery atherosclerosis in hemizygous and heterozygous patients with Fabry disease, compared with a matched control population.
  
  
  METHODS
  The common carotid artery intima-media thickness (IMT) of 53 patients with Fabry disease (24 men, 29 women) was measured by high-definition ultrasonography, and the presence or absence of atherosclerotic plaques reported. Results were compared with those of 120 age-matched healthy individuals (83 men, 37 women).
  
  
  RESULTS
  The common carotid artery IMT was increased to the same extent in male and female patients with Fabry disease (706+/-211 microm and 749+/-395 microm, respectively) compared with that of the control population (614+/-113 microm). In the Fabry population, IMT did not correlate with either systolic blood pressure or with renal function (plasma creatinine). In the control population, only systolic blood pressure was positively and significantly correlated with IMT. Atherosclerotic plaques in the common carotid artery were not observed in any patient with Fabry disease, whereas 34% of the control population had carotid artery plaques, as evidenced by focal non-homogeneous intima-media thickening greater than 1.2 mm.
  
  
  CONCLUSIONS
  This study presents evidence of a major increase in common carotid artery IMT, both in hemizygous and heterozygous patients with Fabry disease, in the absence of focal atherosclerotic plaques. These results suggest that the conduit arteries may be protected from atherosclerosis in Fabry disease.
authors.affiliation: Division of Nephrology, University Hospital, Lausanne, Switzerland.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Barbey
authors.forename: Noureddine
authors.initials: N
authors.lastname: Brakch
authors.forename: Ales
authors.initials: A
authors.lastname: Linhart
authors.forename: Xavier
authors.initials: X
authors.lastname: Jeanrenaud
authors.forename: Thomas
authors.initials: T
authors.lastname: Palecek
authors.forename: Jan
authors.initials: J
authors.lastname: Bultas
authors.forename: Michel
authors.initials: M
authors.lastname: Burnier
authors.forename: Daniel
authors.initials: D
authors.lastname: Hayoz
mesh_terms: D000328:Adult; D002340:Carotid Artery Diseases / Q000000981:diagnostic imaging / Q000473:pathology*; D017536:Carotid Artery, Common / Q000000981:diagnostic imaging / Q000473:pathology; D000795:Fabry Disease / Q000000981:diagnostic imaging / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D017539:Tunica Intima / Q000473:pathology*; D017540:Tunica Media / Q000473:pathology*; D014463:Ultrasonography