ArticleInternational journal of retina and vitreous2022
Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell Disease.
Article in International journal of retina and vitreous, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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15 citing papers in PubMed, 21 citations in OpenAlex.
- Update and review of the current medical and surgical management of sickle cell retinopathy.Eye and vision (London, England) · 2026Review
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- Retinal imaging in an era of open science and privacy protection.Experimental eye research · 2025Review
- Clinical and laboratory risk factors for sickle cell retinopathy and maculopathy: a scoping review of the current evidence.Haematologica · 2025Article
- Sickle cell retinopathy among Ghanaian high school students: a school-based screening.BMJ global health · 2024Article
- Salmon patch maculopathy: An amblyogenic complication of pediatric sickle cell retinopathy.American journal of ophthalmology case reports · 2024Article
- Predictors of health-related quality of life in a large cohort of adult patients living with sickle cell disease in France: the DREPAtient study.Frontiers in public health · 2024Article
- Profiles of systemic lupus erythematosus patients with co-existing sickle cell disease: a coincidence or true association?Reumatologia · 2024Article
- A 21-Year-Old Woman with Sickle Cell Disease and Vaso-Occlusive Pain Associated with Using an Electronic Nicotine Dispensing System (E-Cigarette or Vape) - a Case Report.The American journal of case reports · 2023Article
- Natural history and rate of progression of retinopathy in adult patients with sickle cell disease: an 11-year follow-up study.Blood advances · 2023Article
- Article
- Severe proliferative retinopathy in a patient with sickle cell trait.American journal of ophthalmology case reports · 2023Article
- Automated Quantitative Assessment of Retinal Vascular Tortuosity in Patients with Sickle Cell Disease.Ophthalmology scienceArticle
- Sickle Cell Disease Related Vasculopathies and Early Evaluation in a Pediatric Population.In vivo (Athens, Greece)Article
Corrections and comments
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Authors and funding
13 authors at 6 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundSickle cell retinopathy (SCR) is one of the most important ocular manifestations of sickle cell disease (SCD). This study aims to assess the prevalence of SCR in SCD, identify risk factors for its development and progression to proliferative sickle cell retinopathy (PSCR), and evaluate the potential implications of these results on clinical practice.
methodsThis research is a secondary analysis of patients diagnosed with SCD from the epidemiological, multicenter Cooperative Study of Sickle Cell Disease (CSSCD). We included all patients who completed a full ophthalmic evaluation. We identified clinical and laboratory SCD characteristics associated with SCR using multivariate logistic regression models. Proliferative sickle cell retinopathy (PSCR) was diagnosed according to the Goldberg classification system.
resultsOf the 1904 study participants with SCD who met the inclusion criteria, 953 (50.1%) had retinopathy; of which 642 (67.3%) had bilateral disease. SCR was associated with older age (p < 0.001), history of smoking (p = 0.001), hematuria (p = 0.050), and a lower hemoglobin F (HbF) level (p < 0.001). PSCR risk increased with smoking (p = 0.005), older age (p < 0.001) higher hemoglobin level (p < 0.001) and higher white blood cell count (p = 0.011). Previous blood transfusion (p = 0.050), higher reticulocyte count (p = 0.019) and higher HbF level (p < 0.001) were protective factors against the development of PSCR. Ocular symptoms were associated with progression to PSCR in patients with SCR (p = 0.021).
conclusionIn this cohort of individuals with SCD, half of the participants had signs of SCR. Smoking and blood hemoglobin level were the two modifiable risk factors associated with increased retinopathy progression. Screening to identify the different stages of retinopathy, actively promoting smoking cessation, and optimizing the hematological profile of patients with SCD should guide treatment protocols designed to prevent the vision-threatening complications of the disease.
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