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Updated: Oct 15, 2026
A Workflow to Quantitatively Determine Age-Related Macular Degeneration Lesion-Specific Variations in Fundus Autofluorescence
Published on: May 26, 2023
Progression of Macular Tissue Loss in Choroideremia and Its Association with Baseline Fundus Autofluorescence
Shinichiro Chujo1,2,3, Rouzbeh Abbasgholizadeh1,2, Mai Alhelaly1,2,4
1Doheny Eye Institute, Pasadena, California.
Objective:
To characterize short-term progressive loss of macular preserved autofluorescence (PAF) in choroideremia and to determine how baseline PAF morphology influences subsequent structural decline.
Design:
Post hoc longitudinal analysis of a multicenter prospective natural history cohort (NIGHT study).
Participants:
A total of 106 participants (163 eyes) with genetically confirmed choroideremia and gradable fundus autofluorescence (FAF) imaging at baseline and month 12.
Methods:
Regions of PAF were manually segmented on FAF images and converted into binary masks. Total PAF area and PAF perimeter were quantified at baseline and month 12 using an automated ImageJ based workflow. Associations between baseline characteristics (PAF area, perimeter, foveal status, and PAF region focality) and 12 months PAF change were evaluated using linear mixed effects models accounting for intereye correlation.
Main Outcome Measures:
Change in total macular PAF area over 12 months and its association with baseline PAF region characteristics.
Results:
Mean PAF area decreased significantly from 3.77 ± 4.53 mm2 at baseline to 3.37 ± 4.11 mm2 at month 12 (mean change -0.41 ± 0.57 mm2; P < 0.001). Preserved autofluorescence region perimeter also declined significantly (-1.04 ± 2.56 mm; P < 0.001). In multivariable analysis, larger baseline PAF area (β = -0.071, P < 0.001) and greater baseline PAF region perimeter (β = -0.018, P = 0.003) were independently associated with greater PAF loss. In contrast, foveal preservation (P = 0.197) and PAF region focality (P = 0.569) were not significantly associated with progression.
Conclusions:
Macular PAF area measured by FAF demonstrated significant decline over 12 months in choroideremia and may represent a useful structural endpoint for assessing short-term disease progression. Larger baseline PAF area and perimeter were associated with greater subsequent PAF loss. These findings may improve understanding of disease natural history and inform the design of future clinical trials.
Financial Disclosures:
Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
