ABSTRACT
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), HbSß0-thalassemia, and HbSß+-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/HbSß0/HbSß+ 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.
Subject(s)
Anemia, Sickle Cell , Retinal Diseases , Thalassemia , Humans , Adult , Female , Follow-Up Studies , Retrospective Studies , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/diagnosis , Hemoglobin, Sickle , Thalassemia/complications , Retinal Diseases/etiology , Retinal Diseases/complicationsABSTRACT
BACKGROUND: Research on the disease severity of COVID-19 in patients with rheumatic immune-mediated inflammatory diseases (IMIDs) has been inconclusive, and long-term prospective data on the development of SARS-CoV-2 antibodies in these patients are lacking. METHODS: Adult patients with rheumatic IMIDs from the Amsterdam Rheumatology and Immunology Center, Amsterdam were invited to participate. All patients were asked to recruit their own sex-matched and age-matched control subject. Clinical data were collected via online questionnaires (at baseline, and after 1-4 and 5-9 months of follow-up). Serum samples were collected twice and analysed for the presence of SARS-CoV-2-specific antibodies. Subsequently, IgG titres were quantified in samples with a positive test result. FINDINGS: In total, 3080 consecutive patients and 1102 controls with comparable age and sex distribution were included for analyses. Patients were more frequently hospitalised compared with controls when infected with SARS-CoV-2; 7% vs 0.7% (adjusted OR: 7.33, 95% CI: 0.96 to 55.77). Only treatment with B-cell targeting therapy was independently associated with an increased risk of COVID-19-related hospitalisation (adjusted OR: 14.62, 95% CI: 2.31 to 92.39). IgG antibody titres were higher in hospitalised compared with non-hospitalised patients, and slowly declined with time in similar patterns for patients in all treatment subgroups and controls. INTERPRETATION: We observed that patients with rheumatic IMIDs, especially those treated with B-cell targeting therapy, were more likely to be hospitalised when infected with SARS-CoV-2. Treatment with conventional synthetic disease-modifying antirheumatic drugs (DMARDs) and biological DMARDs other than B-cell targeting agents is unlikely to have negative effects on the development of long-lasting humoral immunity against SARS-CoV-2.