Key result
Sickle cell disease linked to ~1.6 m/s lower cf-PWV vs controls, correlating with clinical complications.
Why the study?
How is arterial stiffness altered in patients with sickle cell disease compared to healthy controls, and is it associated with vascular complications?
Observational (n=4,570)
Yes
How is arterial stiffness altered in patients with sickle cell disease compared to healthy controls, and is it associated with vascular complications?
Absolute Event Rate: 7.5% vs 9.1%
p-value: p=<0.0001
Arterial stiffness parameters (PWV and AI) are significantly altered in sickle cell disease and correlate with clinical vascular complications, suggesting a potential prognostic role.
May reflect distinct vascular biology in sickle cell disease; cross-sectional data leave open prospective prognostic value.
BACKGROUND: Although a blood genetic disease, sickle cell disease (SCD) leads to a chronic vasculopathy with multiple organ involvement. We assessed arterial stiffness in SCD patients and looked for associations between arterial stiffness and SCD-related vascular complications. METHODS: The CADRE (Coeur Artères et Drepanocytose, ie, Heart Arteries and Sickle Cell Disease) study prospectively recruited pediatric and adult SCD patients and healthy controls in Cameroon, Ivory Coast, Gabon, Mali, and Senegal. Patients underwent clinical examination, routine laboratory tests (complete blood count, serum creatinine level), urine albumin/creatinine ratio measure, and a measure of carotid-femoral pulse wave velocity (cf-PWV) and augmentation index (AI) at a steady state. The clinical and biological correlates of cf-PWV and AI were investigated by using a multivariable multilevel linear regression analysis with individuals nested in families further nested in countries. RESULTS: Included were 3627 patients with SCD and 943 controls. Mean cf-PWV was lower in SCD patients (7.5±2.0 m/s) than in controls (9.1±2.4 m/s, P<0.0001), and lower in SS-Sβ(0) than in SC-Sβ(+) phenotypes. AI, corrected for heart rate, increased more rapidly with age in SCD patients and was higher in SCD than in control adults. cf-PWV and AI were independently associated with age, sex, height, heart rate, mean blood pressure, hemoglobin level, country, and hemoglobin phenotype. After adjustment for these correlates, cf-PWV and AI were associated with the glomerular filtration rate and osteonecrosis. AI was also associated with stroke, pulmonary hypertension, and priapism, and cf-PWV was associated with microalbuminuria. CONCLUSIONS: PWV and AI are deeply modified in SCD patients in comparison with healthy controls. These changes are independently associated with a lower blood pressure and a higher heart rate but also with the hemoglobin phenotype. Moreover, PWV and AI are associated with several SCD clinical complications. Their prognostic value will be assessed at follow-up of the patients.
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Ranque et al. (2016) conducted an observational in Sickle cell disease (n=4,570). Sickle cell disease vs. Healthy controls was evaluated on Carotid-femoral pulse wave velocity (cf-PWV) (p=<0.0001). Sickle cell disease was associated with lower mean carotid-femoral pulse wave velocity compared to healthy controls (7.5 vs 9.1 m/s, P<0.0001), which correlated with clinical complications.
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