Monitoring of up to 15\xa0years effects of lipoprotein apheresis on lipids, biomarkers of inflammation, and soluble endoglin in familial hypercholesterolemia patients

J Víšek,J Víšek,M Lánska,C Andrýs,C Andrýs,M Bláha,M Lášticová,K Tripská,M Lášticová,M Bláha,I Najmanová,V Bláha,V Bláha,Ivone Cristina Igreja E Sá,J Duintjer Tebbens,M Vicen,P Nachtigal

doi:10.1186/s13023-021-01749-w

Abstract

BackgroundLipoprotein 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.Results14 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.ConclusionsLA 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.

Highlights

Familial hypercholesterolemia (FH) is an inherited disorder characterized by elevated plasma levels of lowdensity lipoprotein (LDL) cholesterol in the absence ofVíšek et al Orphanet J Rare Dis (2021) 16:110(ARH) with loss of adaptor protein function, encoded by the LDL receptor (LDLR) adaptor protein 1 gene (LDLRAP1) [3]
Despite the significant decrease of lipids and soluble endoglin in the familial hypercholesterolemia (FH) patients treated with Lipoprotein apheresis (LA), we found a significant correlation between soluble endoglin and total cholesterol (TC), LDL cholesterol (LDLC), HDL-C only in 7.1% cases and in apolipoprotein B (apoB) only in 14.3% cases (Table 4)
We proposed that long-term LA treatment improves lipid profile, microinflammation and endothelial dysfunction associated with atherosclerosis in familial hypercholesterolemia patients, suggesting a promising improvement in cardiovascular prognosis in most FH patients

Summary

Introduction

Familial hypercholesterolemia (FH) is an inherited disorder characterized by elevated plasma levels of lowdensity lipoprotein (LDL) cholesterol in the absence ofVíšek et al Orphanet J Rare Dis (2021) 16:110(ARH) with loss of adaptor protein function, encoded by the LDLR adaptor protein 1 gene (LDLRAP1) [3]. Familial hypercholesterolemia (FH) is an inherited disorder characterized by elevated plasma levels of lowdensity lipoprotein (LDL) cholesterol in the absence of. Untreated FH is associated with a markedly increased risk of premature cardiovascular disease depending on the specific molecular defect of LDL cholesterol (LDLC) receptor and coexisting cardiovascular risk factors [4,5,6,7,8]. Lipoprotein apheresis (LA) should be considered as a therapeutic option for patients with severe hypercholesterolemia, who have persistently elevated LDL-C levels, despite pharmacological therapy [9]. 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

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