American journal of ophthalmology · 2025
Natural History of Microperimetry and Optical Coherence Tomography in USH2A-Retinopathy: A Structure-Function Association Study.
СтатьяМногоцентровое исследованиеНазвание и аннотация: Перевод в очереди
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Vincent Ajoy, Liang Wendi, Maguire Maureen G, Duncan Jacque L, Ayala Allison R, Bernstein Paul S, Carroll Joseph, Cheetham Janet K, Durham Todd A, Farsiu Sina, Hoyng Carel B, Huckfeldt Rachel M, Jaffe Glenn J, Loo Jessica, Pennesi Mark E, Sahel José-Alain, Singh Mandeep S, Zemborain Zane Z, Birch David G, Lad Eleonora M, Foundation Fighting Blindness Clinical Consortium Investigator Group
Аннотация
PURPOSE: To describe the natural history of optical coherence tomography (OCT) and mesopic microperimetry (MP) parameters in the Rate of Progression of USH2A-related Retinal Degeneration (RUSH2A) study.
DESIGN: Multicenter, international, prospective, longitudinal natural history study.
METHODS: Study Population: 105 participants with biallelic disease-causing variants in USH2A with a clinical diagnosis of Usher Syndrome or autosomal recessive retinitis pigmentosa who underwent annual testing over 4 years.
MAIN OUTCOME MEASURE(S): Ellipsoid zone (EZ) area and central sub-field thickness (CST) on OCT. Mean sensitivity (MS) and mean pointwise sensitivity at predefined functional transition points (MSFTP) on MP.
RESULTS: A total of 101 and 94 participants met criteria for OCT and MP analysis, respectively. The average EZ area was 3.9 ± 5.3 mm2 at baseline which decreased to 3.3 ± 4.1 mm2 at 4 years (rate of change [ROC] [-0.18 mm2/year]). The average CST decreased from 249.7 ± 35.6 to 244.1 ± 39.3 microns over 4 years (-2.14 microns/year). The average MP MS declined from 6.0 ± 5.1 dB (baseline) to 4.5 ± 4.2 (4 years) (-0.39 dB/year). The MSFTP significantly decreased over time (17.9 dB [baseline] to 10.8 dB [4 years]). Notably, at 4 years, there was a meaningful decrease (≥ 7dB) in MSFTP in 46% of study eyes.
CONCLUSIONS: This study establishes the natural history of key structural and functional parameters in USH2A-retinopathy. The MSFTP is a novel, robust parameter that showed clinically meaningful change over time and is a promising tool to monitor treatment efficacy in clinical trials.
DESIGN: Multicenter, international, prospective, longitudinal natural history study.
METHODS: Study Population: 105 participants with biallelic disease-causing variants in USH2A with a clinical diagnosis of Usher Syndrome or autosomal recessive retinitis pigmentosa who underwent annual testing over 4 years.
MAIN OUTCOME MEASURE(S): Ellipsoid zone (EZ) area and central sub-field thickness (CST) on OCT. Mean sensitivity (MS) and mean pointwise sensitivity at predefined functional transition points (MSFTP) on MP.
RESULTS: A total of 101 and 94 participants met criteria for OCT and MP analysis, respectively. The average EZ area was 3.9 ± 5.3 mm2 at baseline which decreased to 3.3 ± 4.1 mm2 at 4 years (rate of change [ROC] [-0.18 mm2/year]). The average CST decreased from 249.7 ± 35.6 to 244.1 ± 39.3 microns over 4 years (-2.14 microns/year). The average MP MS declined from 6.0 ± 5.1 dB (baseline) to 4.5 ± 4.2 (4 years) (-0.39 dB/year). The MSFTP significantly decreased over time (17.9 dB [baseline] to 10.8 dB [4 years]). Notably, at 4 years, there was a meaningful decrease (≥ 7dB) in MSFTP in 46% of study eyes.
CONCLUSIONS: This study establishes the natural history of key structural and functional parameters in USH2A-retinopathy. The MSFTP is a novel, robust parameter that showed clinically meaningful change over time and is a promising tool to monitor treatment efficacy in clinical trials.