Jasleen Kaur Jolly is an optometrist working for the Nuffield Laboratory of Ophthalmology, University of Oxford and Oxford Eye Hospital. She studied at Cardiff University and the University of Manchester, working across the world in various settings to gain a wide range of experience. She is actively involved in clinical research in the fields of retinal gene therapy, cataract surgery outcomes and low vision. Her main interest is improving outcome measures following gene therapy treatments.


Purpose: We set out to characterize the pattern of fundus auto-fluorescence (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 auto-fluorescence 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 age 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 a small change is likely to be significant. Correlation of patient age and area of residual AF produced a clinically relevant index of expected anatomical disease. Progression is 5-10% of the residual area each year and follows a logarithmic pattern with age (r=0.69, P<0.001). The mean half-life of AF is 9 years. Qualitatively, the pattern of remaining AF was bifocal, with maximal preservation around the fovea (predominantly temporal) with late preservation around the optic disc. Conclusions: The area of residual AF in CHM can be measured reproducibly and shows a distinct pattern of loss. The residual area is inversely correlated to age. The ratio of the two variables may provide useful information regarding the rate of progression for any individual at a point in time.