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Baseline Microperimetry and OCT in the RUSH2A Study: Structure-Function Association and Correlation with Disease Severity

Lad, Eleonora M; Duncan, Jacque L; Liang, Wendi; Maguire, Maureen G; Ayala, Allison R; Audo, Isabelle; Birch, David G; ... Foundation Fighting Blindness Consortium Investigator Group; + view all (2022) Baseline Microperimetry and OCT in the RUSH2A Study: Structure-Function Association and Correlation with Disease Severity. American Journal of Ophthalmology 10.1016/j.ajo.2022.08.013. (In press).

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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 SETTING: 16 clinical sites in Europe and North America STUDY 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 USH2A. OBSERVATION 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.

Type: Article
Title: Baseline Microperimetry and OCT in the RUSH2A Study: Structure-Function Association and Correlation with Disease Severity
Location: United States
DOI: 10.1016/j.ajo.2022.08.013
Publisher version: https://doi.org/10.1016/j.ajo.2022.08.013
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
UCL classification: UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL
URI: https://discovery.ucl.ac.uk/id/eprint/10155265
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