TY - JOUR
T1 - Natural history and rate of progression of retinopathy in adult patients with sickle cell disease
T2 - an 11-year follow-up study
AU - Brandsen, Rajani P.
AU - Diederen, Roselie M. H.
AU - Bakhlakh, Siham
AU - Nur, Erfan
AU - Schlingemann, Reinier O.
AU - Biemond, Bart J.
N1 - Funding Information: The authors thank Stichting Pupil (Pupil foundation) for supporting this work by their grant. Publisher Copyright: © 2023 by The American Society of Hematology. Licensed under.
PY - 2023/7/11
Y1 - 2023/7/11
N2 - 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 analyzed disease progression in 129 patients with SCD with a median follow-up period of 11 years (interquartile range, 8.5-12). Patients were divided in 2 groups. The genotypes hemoglobin SS (HbSS), HbSβ0thalassemia, and HbSβ+-thalassemia were grouped together (n = 83; 64.3%), whereas patients with HbSC (n = 46; 35.7%) were grouped separately. Progression of SCR was observed in 28.7% (37 of 129) of patients. Older age (adjusted odds ratio [aOR], 1.073; 95% confidence interval [CI], 1.024-1.125; P = .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 = .043) were associated with PSCR at end of follow-up. Lack of any SCR at end of follow-up was associated with female sex (aOR, 2.555; 95% CI, 1.101-5.931; P = .029), HbSS/HbSβ0/HbSβ+ genotype (aOR, 3.733; 95% CI, 1.131-12.321; P = .031), and higher HbF levels (aOR, 1.119; 95% CI, 1.007-1.243; P = .037). Differentiated strategies for screening and follow-up of SCR could be considered for patients at low or high risk.
AB - 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 analyzed disease progression in 129 patients with SCD with a median follow-up period of 11 years (interquartile range, 8.5-12). Patients were divided in 2 groups. The genotypes hemoglobin SS (HbSS), HbSβ0thalassemia, and HbSβ+-thalassemia were grouped together (n = 83; 64.3%), whereas patients with HbSC (n = 46; 35.7%) were grouped separately. Progression of SCR was observed in 28.7% (37 of 129) of patients. Older age (adjusted odds ratio [aOR], 1.073; 95% confidence interval [CI], 1.024-1.125; P = .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 = .043) were associated with PSCR at end of follow-up. Lack of any SCR at end of follow-up was associated with female sex (aOR, 2.555; 95% CI, 1.101-5.931; P = .029), HbSS/HbSβ0/HbSβ+ genotype (aOR, 3.733; 95% CI, 1.131-12.321; P = .031), and higher HbF levels (aOR, 1.119; 95% CI, 1.007-1.243; P = .037). Differentiated strategies for screening and follow-up of SCR could be considered for patients at low or high risk.
KW - Adult patients with SCD showed significant progression of SCR during followup
KW - Differentiated strategies for screening
KW - followup of retinopathy could be considered for patients at low or high risk
UR - http://www.scopus.com/inward/record.url?scp=85179470972&partnerID=8YFLogxK
U2 - https://doi.org/10.1182/bloodadvances.2022009147
DO - https://doi.org/10.1182/bloodadvances.2022009147
M3 - Article
C2 - 36897257
SN - 2473-9529
VL - 7
SP - 3080
EP - 3086
JO - Blood
JF - Blood
IS - 13
ER -