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SHR Neuro Cancer Cardio Lipid Metab Microb
Schonbach, EM; Janeschitz-Kriegl, L; Strauss, RW; Cattaneo, MEGV; Fujinami, K; Birch, DG; Cideciyan, AV; Sunness, JS; Weleber, RG; Ip, MS; Sadda, SR; Scholl, HPN.
The Progression of Stargardt Disease Using Volumetric Hill of Vision Analyses Over 24 Months: ProgStar Report No.15
AM J OPHTHALMOL. 2021; 230:
Doi: 10.1016/j.ajo.2021.04.015
Web of Science
PubMed
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- Co-authors Med Uni Graz
- Strauß Rupert
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- Abstract:
- PURPOSE: To report the yearly rate of change in mac-ular function in patients with Stargardt disease type 1 (STGD1) over 24 months and to establish a new volu-metric visual function index for use in clinical trials in-vestigating the efficacy on retinal sensitivity. METHODS: Design: International, multicenter, prospective cohort study with 5 study visits every 6 months over 24 months. Participants: A total of 233 individuals with genetically confirmed STGD1 (>= 1 disease-causing ABCA4 variant). Main Outcome Mea-sures: The total volume (V-TOT) beneath the sensitivity surface of a 3-D model of the hill of vision and mean sensitivity (MS) derived from mesopic microperimetry performed with a white stimulus. Changes of V-TOT over time and its correlation with the ABCA4 genotype and baseline features. RESULTS: At baseline, 440 eyes (233 patients) with a mean (SD) age of 33.7 (15.0) years, mean (SD) visual acuity of 46.08 (16.03) ETDRS letters were analyzed with an average V TOT of 0.91 decibel-steradian (dB-sr) and an MS of 10.73 dB. The overall mean rate of de-crease in sensitivity [95% confidence interval] was 0.077 [0.064, 0.090] dB-sr/y for V TOT and 0.87 [0.72, 1.02] dB/year for MS. The progression rate of V TOT depended on baseline visual function (0.029 dB-sr/year for low and 0.120 dB-sr/year for high baseline V TOT; P <.001) and exhibited a difference in the first vs second year of follow-up (0.065 dB-sr/year vs 0.089 dB-sr/year, respectively; P <.001). The absence of pigmentary abnormalities of the retinal pigment epithelium at baseline was found to be associated with a faster progression rate (P <.001), whereas a significant association with the genotype was not detected (P =.7). CONCLUSION: In STGD1, both microperimetric out-comes demonstrate statistically significant and clinically meaningful changes after relatively short follow-up peri-ods. Volumetric modeling may be useful in future interventional clinical trials that aim to improve retinal sen-sitivity or to slow down its decline and for structure-function correlations. (Am J Ophthalmol 2021;000: 1-11. (c) 2021 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NCND license (http://creativecommons.