Retrospective Analysis of Structural Disease Progression in Retinitis Pigmentosa Utilizing Multimodal Imaging

Retrospective Analysis of Structural Disease Progression in Retinitis Pigmentosa Utilizing Multimodal Imaging

Author Cabral, Thiago Autor UNIFESP Google Scholar
Sengillo, Jesse D. Google Scholar
Duong, Jimmy K. Google Scholar
Justus, Sally Google Scholar
Boudreault, Katherine Google Scholar
Schuerch, Kaspar Google Scholar
Belfort, Rubens, Jr. Autor UNIFESP Google Scholar
Mahajan, Vinit B. Google Scholar
Sparrow, Janet R. Google Scholar
Tsang, Stephen H. Google Scholar
Abstract In this report, we assess the natural progression rate of retinitis pigmentosa (RP) over an average of three years using spectral-domain optical coherence tomography (SD-OCT) and short wavelength fundus autofluorescence (SW-AF). Measurement of the ellipsoid zone (EZ) line width and hyperautofluorescent ring diameters was performed in 81 patients with RP in a retrospective, longitudinal fashion. Rate of structural disease progression, symmetry between eyes, and test-retest variability were quantified. We observed on average, EZ-line widths decreased by 140 mu m (5.2%, p < 0.001) per year, and average horizontal and vertical hyperautofluorescent ring diameters decreased by 149 mu m (3.6%, p < 0.001) and 120 mu m (3.9%, p < 0.001) per year, respectively. The 95th percentile of this cohort had differences in progression slopes between eyes that were less than 154 mu m, 118 mu m, and 132 mu m for EZ-line width and horizontal and vertical ring diameters, respectively. For all measures except horizontal ring diameter, progression rates were significantly slower at end-stage disease. From our data, we observed a statistically significant progression rate in EZ line width and SW-AF ring diameters over time, verifying the utility of these measurements for disease monitoring purposes. Additionally, calculated differences in progression slopes between eyes may prove useful for investigators evaluating the efficacy of unilateral treatments for RP in clinical trials.
xmlui.dri2xhtml.METS-1.0.item-coverage London
Language English
Sponsor National Institutes of Health
National Cancer Institute Core
Research to Prevent Blindness (RPB) Physician-Scientist Award
RPB medical student eye research fellowship
ICO-Retina Research Foundation Helmerich Fellowships - Retina Research Foundation
ICO Foundation
Tistou and Charlotte Kerstan Foundation
Schneeweiss Stem Cell Fund, New York State
Foundation Fighting Blindness New York Regional Research Center
Joel Hoffman Fund
Professor Gertrude Rothschild Stem Cell Foundation
Gebroe Family Foundation
Date 2017
Published in Scientific Reports. London, v. 7, p. -, 2017.
ISSN 2045-2322 (Sherpa/Romeo, impact factor)
Publisher Nature Publishing Group
Extent -
Access rights Open access Open Access
Type Article
Web of Science ID WOS:000408997700046

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