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Robert P. Finger, Manjula Marella, Eva Fenwick, Ecosse L. Lamoureux, Frank G. Holz; The Impact Of Concurrent Vision Loss And Systemic Cardiovascular Disease On Health-related Quality Of Life In Pseudoxanthoma Elasticum. Invest. Ophthalmol. Vis. Sci. 2011;52(14):5560.
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To investigate the impact of pseudoxanthoma elasticum (PXE), a rare hereditary disease of concurrent vision impairment (VI) and cardiovascular complications (CVC), on health-related quality of life (HRQoL).
135 German patients, from the PXE clinic at the department of ophthalmology, University of Bonn, were recruited into the study in 2008. Vision-related QoL was assessed using the Impact of Vision Impairment (IVI) questionnaire and general health with the short form health survey (SF-36). Sociodemographic and clinical data were also collected. Patients were stratified into four categories: group A- no VI and no CVC (n=25); group B- CVC only (n=45); group C- VI only (n=20); and group D- both VI and CVC (n=42). Rasch analysis confirmed two separate constructs for the IVI, namely vision-related functioning and emotional well-being; and the physical and mental summary components of the SF-36.
The mean±SD age and distance visual acuity for the 135 PXE patients (88 women, 66%) were 54±14 years and 20/100 (LogMAR 0.66 ±0.67), respectively. Controlling for age, gender, and general health; vision impaired patients of groups C and D reported poorer visual functioning than groups A and B who did not have any VI (p=0.007). No differences were found in vision-specific functioning and emotional well-being between groups C and D despite the presence of CVC in group D (p=0.342). Group B reported poorer vision-specific emotional well-being compared to groups C and D (p=0.02). Vision-specific emotional well-being did not differ between groups A, C and D (p>0.05). The SF-36 physical and mental summary measures were not significantly different among the groups (p>0.05).
This first study of HRQoL in PXE shows a significant impact on vision-specific functioning and emotional well-being in patients with vision impairment, irrespective of other CVC. Impact on general health was found to be little especially in visually impaired patients. Our results indicate that PXE patients are impacted considerably by vision loss indicating a need for specialized treatment and rehabilitation.
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