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Baseline Microperimetry and OCT in the RUSH2A Study: Structure-Function Association and Correlation With Disease
Eleonora M Lad1, Jacque L Duncan2, Wendi Liang3
1From the Department of Ophthalmology, Duke University Medical Center Durham, North Carolina, USA.
American Journal of Ophthalmology
|August 25, 2022
Summary
Longer disease duration in USH2A-related retinal degeneration correlates with worsening vision and retinal structure. Microperimetry and OCT metrics show significant associations, aiding disease progression insights.
Area of Science:
- Ophthalmology
- Genetics
- Retinal Diseases
Background:
- USH2A gene variants cause Usher syndrome type 2 (USH2) and nonsyndromic autosomal recessive retinitis pigmentosa (ARRP).
- Understanding the natural history of USH2A-related retinal degeneration is crucial for developing effective treatments.
Purpose of the Study:
- To investigate baseline mesopic microperimetry (MP) and spectral domain optical coherence tomography (OCT) in the USH2A-related Retinal Degeneration (RUSH2A) study.
- To establish correlations between retinal structure and function in patients with USH2A variants.
Main Methods:
- A natural history study conducted at 16 clinical sites across Europe and North America.
- Participants included 80 individuals with USH2 and 47 with ARRP associated with USH2A variants.
- General linear models and Spearman correlation coefficients were used to analyze MP (mean sensitivity) and OCT (intact ellipsoid zone area, central subfield thickness) data in relation to disease duration, best corrected visual acuity (BCVA).
Main Results:
- OCT and MP data were obtained from 127 and 93 participants, respectively.
- No significant differences in EZ area, CST, or mean sensitivity were observed between USH2 and ARRP groups.
- Longer disease duration was associated with smaller EZ area and lower mean sensitivity (P < 0.001 and P = 0.01, respectively).
- Greater mean sensitivity correlated with better BCVA, larger EZ area, and larger CST (r > 0.3, P < 0.01).
- Better BCVA and larger CST were associated with larger EZ area (r > 0.6, P < 0.001).
Conclusions:
- Longer disease duration correlates with more severe retinal structure and function abnormalities in USH2A-related retinal degeneration.
- Significant associations exist between microperimetry and optical coherence tomography metrics.
- Monitoring these structure-function relationships is vital for understanding disease mechanisms and progression.
