Clinical and Ophthalmic Factors Associated With the Severity of Sickle Cell Retinopathy

To identify associations between severity of sickle cell retinopathy (SCR) and other clinical, laboratory, or treatment factors relevant to sickle cell disease (SCD).

Retrospective cohort study.

We investigated clinical, laboratory, and demographic associations with the severity of SCR in 296 patients seen at both our SCD specialty clinic and our retina clinic. Multivariate multinomial logistic regression was used to estimate the association between each clinical variable and severity of SCR.

Multivariate analysis showed that in patients with sickle cell anemia (SCA) genotypes, older age (95% confidence interval [CI], 1.04-1.15; P < .001) and male sex (95% CI, 0.13-0.87; P = .02) were associated with proliferative sickle cell retinopathy (PSR). In patients with genotypic variants, visual symptoms (95% CI, 1.36-21.62; P = .02) were associated with PSR. Laser photocoagulation and vitrectomy surgery, the standard interventions for PSR, were associated with older age (95% CI, 1.05-1.13; P < .001), visual symptoms (95% CI, 1.48-7.40; P = .004), higher hemoglobin level (95% CI, 1.14-1.65; P = .001), and no chronic transfusion (95% CI, 0.16-1.09; P = .08) across the whole cohort.

These findings may inform clinicians of the symptoms, systemic findings, and disease-modifying therapies most frequently associated with SCR in SCD patients. Visual symptoms such as blurred vision or floaters were associated with progression of SCR and may be criteria for referral for retinal examination. Chronic transfusion therapy may be protective against the need for retinal laser photocoagulation or vitrectomy. Prospective studies are necessary to further explore risk factors for SCR and to identify which individuals with SCD are at risk for incident or progression of retinopathy.