Baseline Microperimetry and OCT in the RUSH2A Study: Structure−Function Association and Correlation With Disease Severity - 25/11/22
, Isabelle Audo d, e, David G. Birch f, Joseph Carroll g, Janet K. Cheetham h, Todd A. Durham h, Abigail T. Fahim i, Jessica Loo j, Zengtian Deng j, Dibyendu Mukherjee j, Elise Heon k, Robert B. Hufnagel l, Bin Guan l, Alessandro Iannaccone a, Glenn J. Jaffe a, Christine N. Kay m, Michel Michaelides n, Mark E. Pennesi o, Ajoy Vincent k, Christina Y. Weng p, Sina Farsiu a, jfor the
Foundation Fighting Blindness Consortium Investigator Group
Highlights |
• | Baseline microperimetry and spectral domain OCT were analyzed in the RUSH2A study. |
• | Better best corrected visual acuity and larger subfield thickness were associated with larger ellipsoid zone area. |
• | Longer disease duration correlated with more severe structure−function abnormalities. |
• | Monitoring these changes will provide important insights into disease mechanisms. |
Abstract |
Purpose |
To investigate baseline mesopic microperimetry (MP) and spectral domain optical coherence tomography (OCT) in the Rate of Progression in USH2A-related Retinal Degeneration (RUSH2A) study.
Design |
Natural history study
Methods |
Setting: 16 clinical sites in Europe and North AmericaStudy Population: Participants with Usher syndrome type 2 (USH2) (N = 80) or autosomal recessive nonsyndromic RP (ARRP) (N = 47) associated with biallelic disease-causing sequence variants in USH2AObservation Procedures: General linear models were used to assess characteristics including disease duration, MP mean sensitivity and OCT intact ellipsoid zone (EZ) area. The associations between mean sensitivity and EZ area with other measures, including best corrected visual acuity (BCVA) and central subfield thickness (CST) within the central 1 mm, were assessed using Spearman correlation coefficients.Main Outcome Measures: Mean sensitivity on MP; EZ area and CST on OCT.
Results |
All participants (N = 127) had OCT, while MP was obtained at selected sites (N = 93). Participants with Usher syndrome type 2 (USH2, N = 80) and nonsyndromic autosomal recessive Retinitis Pigmentosa (ARRP, N = 47) had the following similar measurements: EZ area (median (interquartile range [IQR]): 1.4 (0.4, 3.1) mm2 vs 2.3 (0.7, 5.7) mm2) and CST (median (IQR): 247 (223, 280) µm vs 261 (246, 288), and mean sensitivity (median (IQR): 3.5 (2.1, 8.4) dB vs 5.1 (2.9, 9.0) dB). Longer disease duration was associated with smaller EZ area (P < 0.001) and lower mean sensitivity (P = 0.01). Better BCVA, larger EZ area, and larger CST were correlated with greater mean sensitivity (r > 0.3 and P < 0.01). Better BCVA and larger CST were associated with larger EZ area (r > 0.6 and P < 0.001).
Conclusions |
Longer disease duration correlated with more severe retinal structure and function abnormalities, and there were associations between MP and OCT metrics. Monitoring changes in retinal structure-function relationships during disease progression will provide important insights into disease mechanism in USH2A-related retinal degeneration.
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| Meeting Presentation: Parts of the data in this manuscript were presented at the Retina Society 2020, the Macula Society 2021, and ARVO 2021. |
Vol 244
P. 98-116 - décembre 2022 Retour au numéro
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