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Meta-Analysis
. 2022 Jan 16;22(1):29.
doi: 10.1186/s12886-022-02250-z.

Bilateral visual acuity decline in males with choroideremia: a pooled, cross-sectional meta-analysis

Duygu Bozkaya  1 , Heng Zou  2 , Cindy Lu  2 , Nicole W Tsao  2 , Byron L Lam  3
Affiliations
Meta-Analysis

Bilateral visual acuity decline in males with choroideremia: a pooled, cross-sectional meta-analysis

Duygu Bozkaya et al. BMC Ophthalmol. .

Abstract

Background: Choroideremia is a rare inherited retinal disease that leads to blindness. Visual acuity (VA) is a key outcome measure in choroideremia treatment studies, but VA decline rates change with age. An accurate understanding of the natural deterioration of VA in choroideremia is important to assess the treatment effect of new therapies in which VA is the primary outcome measure. We conducted a meta-analysis of data on individuals with choroideremia to determine the rate of VA deterioration between the better- and worse-seeing eye (BSE and WSE, respectively).

Methods: Data were collected from the prospective Natural History of the Progression of Choroideremia (NIGHT) study (613 eyes, baseline data only), studies included in a recent meta-analysis, and studies identified in a targeted literature search performed on March 25, 2020, including individual best-corrected VA (BCVA) and age data in male individuals with choroideremia. Best-corrected VA decline rates (measured by logMAR units) by age and trends in BCVA decline rates in the BSE and WSE were evaluated. RESULTS: Data from 1037 males (1602 eyes; mean age, 41.8 years) were included. Before and after an age cutoff of 33.8 years, BCVA decline rates for the WSE were 0.0086 and 0.0219 logMAR per year, respectively. Before and after an age cutoff of 39.1 years, BCVA decline rates for the BSE were 0.00001 and 0.0203 logMAR per year, respectively. Differences in absolute BCVA and decline rates increased between the 2 eyes until age ~ 40; thereafter, differences in absolute BCVA and decline rates were similar between eyes.

Conclusions: Using the largest choroideremia data set to date, this analysis demonstrates accelerated BCVA decline beginning between 30 and 40 years of age. Disparate interocular progression rates were observed before the transition age, with similar interocular progression rates after the transition age.

Keywords: Bilateral; Eye symmetry; Interocular; Retinal disease; Visual acuity.

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Conflict of interest statement

DB, HZ, and CL are employees of Biogen and may hold stock in the company. NWT is an employee of Sanofi Genzyme and was an employee of Biogen at the time of the study and may hold stock in the company. BLL has served as a consultant for Biogen and ProQR Therapeutics and has received research and clinical trial funding from Applied Genetic Technologies Corporation, Biogen, Editas Medicine, National Eye Institute, Pixium Vision, and ProQR Therapeutics.

Figures

Fig. 1
Fig. 1
Mean BCVA (logMAR) by age groups with all available data in (A) both eyes, (B) worse-seeing eyes, and (C) better-seeing eyes. BCVA best-corrected visual acuity
Fig. 2
Fig. 2
BCVA (logMAR) category distribution by age at baseline in (A) both eyes, (B) worse-seeing eyes, and (C) better-seeing eyes. BCVA best-corrected visual acuity
Fig. 3
Fig. 3
BCVA (logMAR) over time in WSE and BSE by age in (A) all individuals and (B) all individuals excluding those in the NIGHT study. Individual data points are represented, along with the segmented regression trendlines before and after the transition age in the BSE (black and blue lines, respectively) and WSE (lavender and green lines, respectively). BCVA best-corrected visual acuity, BSE better-seeing eye, WSE worse-seeing eye
Fig. 4
Fig. 4
Interocular asymmetry based on mean difference in BCVA (logMAR) score between eyes by age group in all individuals (blue) and in all individuals excluding those in the NIGHT study (green). Overall trendline for all data (blue line) is shown. Error bars represent SD. BCVA best-corrected visual acuity
See this image and copyright information in PMC

References

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