This retrospective cohort study evaluated progression rates and risk factors for sickle cell retinopathy (SCR) in 317 patients (620 eyes) seen at the Wilmer Eye Institute between 2013–2023, with median follow-up of 7.8 years (non-proliferative) and 11.1 years (proliferative).
Progression occurred in 10.7% of baseline non-proliferative SCR eyes and 17.0% of baseline proliferative SCR eyes. Active smoking at PSR diagnosis was the only significant predictor of progression to more advanced PSR (HR 2.78, 95% CI 1.50–5.16; q=0.005). Age, sex, genotype, hydroxyurea use, transfusions, red cell exchanges, bone marrow transplantation, and macular OCT findings were not associated with progression.
- Retrospective design with potential selection bias from a single tertiary center (Wilmer Eye Institute), limiting generalizability. - Relatively small proportion of eyes progressed, which may have limited power to detect associations with rarer risk factors. - OCT assessment was qualitative rather than quantitative, which may have reduced sensitivity for detecting structural correlates of progression.
Counsel all patients with proliferative sickle cell retinopathy to stop smoking — active smoking nearly triples the risk of progression. Establish early ophthalmic referral and long-term surveillance for PSR patients, as nearly 1 in 5 progressed over a median of 11 years.
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