title: The relative impact of vision impairment and cardiovascular disease on quality of life: the example of pseudoxanthoma elasticum.
abstract: OBJECTIVE
  To investigate the impact of pseudoxanthoma elasticum (PXE), a rare hereditary disease of concurrent vision impairment (VI) and cardiovascular complications (CVCs), on vision-related (VRQoL) and health-related quality of life (HRQoL).
  
  
  METHODS
  VRQoL and HRQoL were assessed using the Impact of Vision Impairment (IVI) questionnaire and the Short Form Health Survey (SF-36) in 107 PXE patients. Patients were stratified into four groups: A = no VI or CVC; B = CVCs only; C = VI only; and D = both VI and CVCs.
  
  
  RESULTS
  Following Rasch analysis, the IVI was found to function as a vision-specific functioning and emotional well-being subscale, and the SF-36 as a health-related physical functioning and mental health subscale. The presence of VI and CVC were significant predictors of vision-specific functioning and emotional well-being (p < 0.001), with a clinically meaningful decrement in vision-specific functioning in patients with VI. No associations were found for the SF-36 Physical Functioning and Mental Health scores between any groups.
  
  
  CONCLUSIONS
  Vision impaired patients with PXE report significantly poorer vision-specific functioning than PXE patients without VI. In contrast, the relative impact of PXE on reported general HRQoL was much less. Our results suggest that vision impairment has the larger impact on QoL in this sample.
authors.affiliation: Department of Ophthalmology, University of Bonn, Ernst-Abbe-Strasse 2, D-53127, Bonn, Germany. robertfinger@gmx.net
authors.forename: Robert P
authors.initials: RP
authors.lastname: Finger
authors.forename: Eva
authors.initials: E
authors.lastname: Fenwick
authors.forename: Manjula
authors.initials: M
authors.lastname: Marella
authors.forename: Peter
authors.initials: P
authors.lastname: Charbel Issa
authors.forename: Hendrik P N
authors.initials: HP
authors.lastname: Scholl
authors.forename: Frank G
authors.initials: FG
authors.lastname: Holz
authors.forename: Ecosse L
authors.initials: EL
authors.lastname: Lamoureux
mesh_terms: D000328:Adult; D002318:Cardiovascular Diseases / Q000150:complications* / Q000175:diagnosis / Q000523:psychology; D005260:Female; D006304:Health Status; D006801:Humans; D008297:Male; D011561:Pseudoxanthoma Elasticum / Q000150:complications* / Q000175:diagnosis / Q000523:psychology; D011594:Psychometrics; D011788:Quality of Life; D014786:Vision Disorders / Q000150:complications* / Q000175:diagnosis / Q000523:psychology