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Martin Gliem, Philipp L. Müller, Johannes Birtel, Myra B. McGuinness, Robert P. Finger, Philipp Herrmann, Doris Hendig, Frank G. Holz, Peter Charbel Issa; Quantitative Fundus Autofluorescence in Pseudoxanthoma Elasticum. Invest. Ophthalmol. Vis. Sci. 2017;58(14):6159-6165. doi: 10.1167/iovs.17-22007.
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To quantify lipofuscin-associated fundus autofluorescence in patients with pseudoxanthoma elasticum (PXE), a model disease for Bruch's membrane pathology.
In this prospective, monocenter, cross-sectional case-control study, 49 patients with PXE (mean age: 46 years, range 18–62) underwent quantitative fundus autofluorescence (qAF) imaging with a modified scanning laser ophthalmoscope containing an internal fluorescent reference for normalization of images. The mean qAF values of a circular ring centered on the fovea (qAF8) were measured and compared to 108 healthy controls (mean age 40 years, range 18–64).
Overall, patients with PXE showed lower qAF8 values compared to controls (difference from controls −23%, 95% confidence interval [CI] −29% to −16%, P < 0.001). The reduction was most pronounced in patients older than 40 years (−30%, 95% CI −36% to −23%, P < 0.001) and was negatively correlated with the extent of Bruch's membrane calcification (r = −0.49, 95% CI: −0.67 to −0.22). The topographic distribution revealed a greater reduction of qAF values toward the optic disc than temporally compared to controls (P < 0.001). The phenotype of patients with reduced qAF values was characterized by pattern-dystrophy-like changes (71%; 10 of 14), reticular pseudodrusen (71%; 10 of 14) and limited areas of atrophy (29%, 4 of 14).
Reduced qAF8 values are a characteristic finding in patients with PXE, indicating that Bruch's membrane disease may result in a modification of the accumulation, distribution, or composition (or a combination thereof) of lipofuscin in retinal pigment epithelial cells.
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