Natural history and rate of progression of retinopathy in adult sickle cell disease patients: an 11-year follow-up study.

Sickle cell retinopathy (SCR) is a complication of sickle cell disease (SCD). Proliferative SCR (PSCR) can lead to severe visual impairment due to vitreous hemorrhage or retinal detachment. Knowledge of risk factors for progression and complications of SCR is limited. The aim of this study is to describe the natural history of SCR and to identify risk factors for progressive SCR and development of PSCR. We retrospectively analysed disease progression in 129 SCD patients with a median follow-up period of 11 years (IQR = 8.5-12). Patients were divided in two groups. The genotypes HbSS, HbSβ0-thalassemia and HbSβ+-thalassemia were grouped together (n=83, 64.3%), while patients with HbSC (n=46, 35.7%) were grouped separately. Progression of SCR was observed in 28.7% (37/129). Age (aOR: 1.073, 95% CI: 1.024-1.125, p = 0.003), HbSC genotype (aOR: 25.472, 95% CI: 3.788-171.285, p = <0.001) and lower HbF (aOR: 0.786, 95% CI: 0.623-0.993, p = 0.043) were associated with PSCR at end of follow-up. Lack of any SCR at end of follow-up was associated with female gender (aOR: 2.555, 95% CI: 1.101-5.931, p = 0.029), HbSS/HbSβ0/HbSβ+ genotype (aOR: 3.733, 95% CI: 1.131-12.321, p = 0.031) and higher HbF levels (aOR: 1.119, 95% CI: 1.007-1.243, p = 0.037). Differentiated strategies for screening and follow-up of SCR could be considered for these low-risk and high-risk patients.