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Purpose: We set out to characterize the pattern of fundus autofluorescence (AF) loss in choroideremia (CHM) patients of varying ages and disease severity in order to determine the average rate of progression of this potential disease biomarker. Methods: Fifty consecutive CHM patients (100 eyes) attending outpatient clinics at Oxford Eye Hospital underwent analysis with the Heidelberg OCT Spectralis with autofluorescence capabilities. The area of residual AF was traced using Heidelberg Eye Explorer. Bland-Altman analysis was used to calculate the coefficient of repeatability (CR). The degree of AF loss was correlated to different ages and the pattern of residual AF constructed into color-coded maps in order to gain insight into the mechanism of disease progression. Results: The CR for measurement of AF area is <1%, indicating that a small change is likely to be significant. Correlation of patient age and area of residual AF produced a clinically relevant index of expected anatomic disease. Progression is 7.7% of the residual area each year (95% confidence intervals 7.0%-8.2%) and follows a logarithmic pattern with age (r = 0.95, P < 0.001). From this we derived the mean half-life of AF as 9 years. Qualitatively, the pattern of remaining AF centered on a point temporal to the fovea. Conclusions: The area of residual AF in CHM can be measured reproducibly and shows a distinct pattern of loss. The measured residual area is inversely correlated to age. The ratio of the two variables may provide useful information regarding the rate of progression for any one individual at a given point in time.

Original publication

DOI

10.1167/iovs.15-18362

Type

Journal article

Journal

Invest Ophthalmol Vis Sci

Publication Date

01/08/2016

Volume

57

Pages

4498 - 4503

Keywords

Adolescent, Adult, Aged, Choroideremia, Disease Progression, Female, Fluorescein Angiography, Fluorescence, Fovea Centralis, Fundus Oculi, Humans, Male, Middle Aged, Ophthalmoscopy, Reproducibility of Results, Severity of Illness Index, Tomography, Optical Coherence, Visual Acuity, Young Adult