Monitoring of up to 15 years effects of lipoprotein apheresis on lipids, biomarkers of inflammation, and soluble endoglin in familial hypercholesterolemia patients

Abstract

Background Lipoprotein apheresis (LA) is considered as an add-on therapy for patients with familial hypercholesterolemia (FH). We aimed to analyze the data collected in the last 15 years from FH patients treated with LA, to elucidate the benefit of this procedure with respect to plasma lipids, biomarkers of inflammation, and endothelial dysfunction and soluble endoglin. Results 14 patients (10 heterozygous FH patients (HeFH), 4 homozygous FH patients (HoFH)) were treated by long-term lipoprotein apheresis. Lipid levels were examined, and ELISA detected biomarkers of inflammation and soluble endoglin. Paired tests were used for intergroup comparisons, and a linear regression model served to estimate the influence of the number of days patients were treated with LA on the studied parameters. LA treatment was associated with a significant decrease of total cholesterol (TC), LDL-C, HDL-C, and apoB, in both HeFH and HoFH patients, after single apheresis and in a long-term period during the monitored interval of 15 years. Biomarkers of inflammation and endothelial dysfunction were reduced for soluble endoglin, hsCRP, and MCP-1, and sP-selectin after each procedure in some HeFH and HoFH patients. Conclusions LA treatment up to 15 years, reduced cholesterol levels, levels of biomarkers related to endothelial dysfunction, and inflammation not only after each procedure but also in the long-term evaluation in FH patients. We propose that long-term LA treatment improves lipid profile and endothelial dysfunction in familial hypercholesterolemia patients, suggesting a promising improvement in cardiovascular prognosis in most FH patients.

Efficacy and Safety of Lomitapide in Homozygous Familial Hypercholesterolaemia: A Systematic Review

“…J. Višek et al . [ 42 ] analyzed data on FH patients treated with LA for 15 years. They found that long-term LA treatment improved lipid levels and endothelial dysfunction, without cardiovascular complications.…”

Soluble endoglin reduces thrombus formation and platelet aggregation via interaction with αIIbβ3 integrin

“…Furthermore, the pathogenic role of sEng in preeclampsia-associated hypertension and renal involvement has been demonstrated in several in vivo studies [9,[18][19][20]. Abnormal high levels of sEng are also found in other cardiovascular-related conditions such as atherosclerosis, hypercholesterolemia [21,22], diabetes mellitus [23][24][25], diabetic retinopathy [26], hypertension [23], circulatory failure in septic shock syndrome [27], coronary artery disease [28][29][30], acute myocardial infarction [31], and reperfusion after cerebral large-vessel occlusion [32]. These findings are in agreement with several in vivo and in vitro studies supporting an active role of sEng in endothelial dysfunction and vascular remodeling [33][34][35].…”

Clinical practice recommendations on lipoprotein apheresis for children with homozygous familial hypercholesterolemia: an expert consensus statement from ERKNet and ESPN

“…3, 4 LA has to be repeated weekly or biweekly, as LDL-C levels increase after each session. 3, 5 Although the lipid-lowering effect of LA is strong, its impact on preventing ASCVD in HoFH is difficult to analyse. HoFH is rare, LA is not available for many patients around the globe and if available, it is not ethical to withhold children from LA to analyse the clinical efficacy.…”