Abstract
Background/Objectives
To assess systemic associations of angioid streaks (AS) using a large US healthcare database.
Subjects/Methods
A retrospective cross-sectional study was conducted of patients diagnosed with AS in a large, national US insurer from 2000–2019. Cases were matched 1:5 to controls. The prevalence rates of established associated disease states and other systemic diseases were calculated and compared using logistic regression. Additionally, the rate of anti-VEGF treatment was assessed as a proxy for the incidence of choroidal neovascularization (CNV).
Results
One thousand eight hundred fifty-two cases of AS and 9028 matched controls were included. The rates of association between AS and the well-characterized conditions included: Pseudoxanthoma elasticum (PXE)—228 patients (12.3%), Ehlers–Danlos syndrome—18 patients (1.0%), Paget’s disease—6 patients (0.3%), hemoglobinopathies—30 patients (1.6%), and idiopathic—1573 patients (84.9%). There was a statistically higher prevalence of the following less classically associated diseases among patients with AS compared to controls: hereditary spherocytosis (1.7% vs. 0.6%, p < 0.001), connective tissue disease (1.0% vs 0.3%, p < 0.001) and non-exudative age-related macular degeneration (33.9% vs 10.6%, p < 0.001). Among 1442 eligible cases analyzed, 427 (29.6%) received at least 1 anti-VEGF injection with 338 (23.4%) patients having the injection after their AS diagnosis.
Conclusions
In the largest collection of AS patients to date, the classical teaching of systemic disease associations occur at rates far, far lower than previously reported. The association of AS with other less reported diseases highlights new potential associations and may contribute to the understanding of AS formation.
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Data availability
Data for this study were licensed from OptumInsight (Eden Park, MN, USA) by the University of Pennsylvania and due to contractual obligations are not permitted to be made freely available. It is available for licensing directly from the company.
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Funding
National Institutes of Health K23 Award (1K23EY025729 - 01) and University of Pennsylvania Core Grant for Vision Research (2P30EY001583). The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH. Additional funding was provided by Research to Prevent Blindness and the Paul and Evanina Mackall Foundation. Funding from each of the above sources was received in the form of block research grants to the Scheie Eye Institute. None of the organizations had any role in the design or conduction of the study.
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Study idea: JN, YL. Study design: BM, BVB. Study conduction: BM, BVB. Results analysis: JN, YL BVB. Manuscript drafting: JN, YL, BVB. Critical revision of manuscript: JN, YL, BM, BVB.
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Nadelmann, J.B., Li, Y., McGeehan, B. et al. Systemic disease associations with angioid streaks in a large healthcare claims database. Eye 37, 1596–1601 (2023). https://doi.org/10.1038/s41433-022-02189-x
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DOI: https://doi.org/10.1038/s41433-022-02189-x