The extent of angioid streaks correlates with macular degeneration in pseudoxanthoma elasticum.

Abstract Purpose To investigate whether the extent of Bruch’s membrane (BM) calcification is associated with choroidal neovascularization (CNV) and macular atrophy in patients with pseudoxanthoma elasticum (PXE) by using the extent of angioid streaks as a surrogate marker for the degree of BM calcification. Design; Retrospective cross-sectional study. Methods We investigated 301 PXE patients (median age 52 years, range 9–79) in a tertiary referral center. For both eyes, we graded the extent of angioid streaks, i.e. their distance from the optic disk, into five groups. Imaging was systematically assessed for signs of CNV and macular atrophy. Associations between extent of angioid streaks and CNV or macular atrophy were investigated using regression analysis. Results CNV was present in 148 patients (49%) and retinal atrophy in 71 patients (24%). The extent of angioid streaks was associated with older age (P for trend = 1.92×10-15) and a higher prevalence of CNV and/or macular atrophy (P for trend = 4.22×10-10 and P for trend = 5.17×10-6, respectively). In addition, the extent of angioid streaks was associated with the presence of CNV when adjusted for age and sex (odds ratio (OR) 1.9, 95% CI 1.3 - 2.9) and with more severe macular atrophy (proportional OR 2.3, 95% CI 1.5 - 3.6). Conclusions In PXE patients, longer angioid streaks are associated with an increased risk of CNV and macular atrophy, even after adjustment for age. These findings are relevant when counseling PXE patients on their visual prognosis.