Differential disease progression in atrophic age-related macular degeneration and late-onset stargardt disease
<p>Purpose: To compare the disease course of retinal pigment epithelium (RPE) atrophy secondary to age-related macula degeneratio (AMD) and late-onset Stargardt disease (STGD1).</p><p> Methods: Patients were examined longitudinally by fundus autofluorescence, near-infrared reflecta...
| Main Authors: | , , , , , , , , , , , , |
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| Format: | Journal article |
| Language: | English |
| Published: |
Association for Research in Vision and Ophthalmology
2017
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| _version_ | 1826269755408908288 |
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| author | Lindner, M Lambertus, S Mauschitz, M Bax, N Kersten, E Lüning, A Nadal, J Schmitz-Valckenberg, S Schmid, M Holz, F Hoyng, C Fleckenstein, M Foveal sparing Atrophy Study Team (FAST) |
| author_facet | Lindner, M Lambertus, S Mauschitz, M Bax, N Kersten, E Lüning, A Nadal, J Schmitz-Valckenberg, S Schmid, M Holz, F Hoyng, C Fleckenstein, M Foveal sparing Atrophy Study Team (FAST) |
| author_sort | Lindner, M |
| collection | OXFORD |
| description | <p>Purpose: To compare the disease course of retinal pigment epithelium (RPE) atrophy secondary to age-related macula degeneratio (AMD) and late-onset Stargardt disease (STGD1).</p><p> Methods: Patients were examined longitudinally by fundus autofluorescence, near-infrared reflectance imaging, and best-corrected visual acuity (BCVA). Areas of RPE atrophy were quantified using semi-automated software, and the status of the fovea was evaluated based on autofluorescence and near-infrared reflectance images. Mixed-effects models were used to compare atrophy progression rates. BCVA loss and loss of foveal integrity were analyzed using Turnbull's estimator.</p><p> Results: A total of 151 patients (226 eyes) with RPE atrophy secondary to AMD and 38 patients (66 eyes) with RPE atrophy secondary to late-onset STGD1 were examined for a median time of 2.3 years (interquartile range, 2.7). Mean baseline age was 74.2 years (SD, 7.6) in AMD and 63.4 (SD, 9.9) in late-onset STGD1 (P = 1.1 × 10-7). Square root atrophy progression was significantly faster in AMD when compared with late-onset STGD1 (0.28 mm/year [SE, 0.01] vs. 0.23 [SE, 0.03]; P = 0.030). In late-onset STGD1, the median survival of the fovea was significantly longer when compared with eyes with AMD (8.60 vs. 3.35 years; P = 0.005) with a trend to a later BCVA loss of ≥3 lines (5.97 vs. 4.37 years; P = 0.382).</p><p> Conclusions: These natural history data indicate differential disease progression in AMD versus late-onset STGD1. The results underline the relevance of refined phenotyping in elderly patients presenting with RPE atrophy in regard to prognosis and design of interventional trials.</p> |
| first_indexed | 2024-03-06T21:30:05Z |
| format | Journal article |
| id | oxford-uuid:4467394b-6e0e-4a65-bca7-d78c71642dfe |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-06T21:30:05Z |
| publishDate | 2017 |
| publisher | Association for Research in Vision and Ophthalmology |
| record_format | dspace |
| spelling | oxford-uuid:4467394b-6e0e-4a65-bca7-d78c71642dfe2022-03-26T15:01:21ZDifferential disease progression in atrophic age-related macular degeneration and late-onset stargardt diseaseJournal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:4467394b-6e0e-4a65-bca7-d78c71642dfeEnglishSymplectic Elements at OxfordAssociation for Research in Vision and Ophthalmology2017Lindner, MLambertus, SMauschitz, MBax, NKersten, ELüning, ANadal, JSchmitz-Valckenberg, SSchmid, MHolz, FHoyng, CFleckenstein, MFoveal sparing Atrophy Study Team (FAST)<p>Purpose: To compare the disease course of retinal pigment epithelium (RPE) atrophy secondary to age-related macula degeneratio (AMD) and late-onset Stargardt disease (STGD1).</p><p> Methods: Patients were examined longitudinally by fundus autofluorescence, near-infrared reflectance imaging, and best-corrected visual acuity (BCVA). Areas of RPE atrophy were quantified using semi-automated software, and the status of the fovea was evaluated based on autofluorescence and near-infrared reflectance images. Mixed-effects models were used to compare atrophy progression rates. BCVA loss and loss of foveal integrity were analyzed using Turnbull's estimator.</p><p> Results: A total of 151 patients (226 eyes) with RPE atrophy secondary to AMD and 38 patients (66 eyes) with RPE atrophy secondary to late-onset STGD1 were examined for a median time of 2.3 years (interquartile range, 2.7). Mean baseline age was 74.2 years (SD, 7.6) in AMD and 63.4 (SD, 9.9) in late-onset STGD1 (P = 1.1 × 10-7). Square root atrophy progression was significantly faster in AMD when compared with late-onset STGD1 (0.28 mm/year [SE, 0.01] vs. 0.23 [SE, 0.03]; P = 0.030). In late-onset STGD1, the median survival of the fovea was significantly longer when compared with eyes with AMD (8.60 vs. 3.35 years; P = 0.005) with a trend to a later BCVA loss of ≥3 lines (5.97 vs. 4.37 years; P = 0.382).</p><p> Conclusions: These natural history data indicate differential disease progression in AMD versus late-onset STGD1. The results underline the relevance of refined phenotyping in elderly patients presenting with RPE atrophy in regard to prognosis and design of interventional trials.</p> |
| spellingShingle | Lindner, M Lambertus, S Mauschitz, M Bax, N Kersten, E Lüning, A Nadal, J Schmitz-Valckenberg, S Schmid, M Holz, F Hoyng, C Fleckenstein, M Foveal sparing Atrophy Study Team (FAST) Differential disease progression in atrophic age-related macular degeneration and late-onset stargardt disease |
| title | Differential disease progression in atrophic age-related macular degeneration and late-onset stargardt disease |
| title_full | Differential disease progression in atrophic age-related macular degeneration and late-onset stargardt disease |
| title_fullStr | Differential disease progression in atrophic age-related macular degeneration and late-onset stargardt disease |
| title_full_unstemmed | Differential disease progression in atrophic age-related macular degeneration and late-onset stargardt disease |
| title_short | Differential disease progression in atrophic age-related macular degeneration and late-onset stargardt disease |
| title_sort | differential disease progression in atrophic age related macular degeneration and late onset stargardt disease |
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