Quantitative Fundus Autofluorescence in Pseudoxanthoma Elasticum
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Quantitative Fundus Autofluorescence in Pseudoxanthoma Elasticum. / Gliem, Martin; Müller, Philipp L; Birtel, Johannes; McGuinness, Myra B; Finger, Robert P; Herrmann, Philipp; Hendig, Doris; Holz, Frank G; Charbel Issa, Peter.
in: INVEST OPHTH VIS SCI, Jahrgang 58, Nr. 14, 01.12.2017, S. 6159-6165.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Quantitative Fundus Autofluorescence in Pseudoxanthoma Elasticum
AU - Gliem, Martin
AU - Müller, Philipp L
AU - Birtel, Johannes
AU - McGuinness, Myra B
AU - Finger, Robert P
AU - Herrmann, Philipp
AU - Hendig, Doris
AU - Holz, Frank G
AU - Charbel Issa, Peter
PY - 2017/12/1
Y1 - 2017/12/1
N2 - PURPOSE: To quantify lipofuscin-associated fundus autofluorescence in patients with pseudoxanthoma elasticum (PXE), a model disease for Bruch's membrane pathology.METHODS: 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).RESULTS: 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).CONCLUSIONS: 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.
AB - PURPOSE: To quantify lipofuscin-associated fundus autofluorescence in patients with pseudoxanthoma elasticum (PXE), a model disease for Bruch's membrane pathology.METHODS: 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).RESULTS: 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).CONCLUSIONS: 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.
KW - Adolescent
KW - Adult
KW - Aged
KW - Cross-Sectional Studies
KW - Female
KW - Fluorescein Angiography/methods
KW - Fundus Oculi
KW - Humans
KW - Male
KW - Middle Aged
KW - Ophthalmoscopy
KW - Prospective Studies
KW - Pseudoxanthoma Elasticum/diagnosis
KW - Retinal Pigment Epithelium/pathology
KW - Tomography, Optical Coherence/methods
KW - Young Adult
U2 - 10.1167/iovs.17-22007
DO - 10.1167/iovs.17-22007
M3 - SCORING: Journal article
C2 - 29214314
VL - 58
SP - 6159
EP - 6165
JO - INVEST OPHTH VIS SCI
JF - INVEST OPHTH VIS SCI
SN - 0146-0404
IS - 14
ER -
