Serum cholesterol is commonly elevated in patients with PBC, alike other cholestatic conditions. The increased cholesterol level in PBC is largely caused by the presence of LpX [32] . LpX is an abnormal lipid particle which is characteristic for cholestatic liver disease and that is directly derived from biliary lipids that regurgitate into the blood [33] . Upon lipoprotein fractionation, LpX is usually found in the very low-density lipoprotein fraction but is very different from other lipoproteins. In contrast to normal lipoproteins, which have a core filled with neutral lipid, LpX consists of liposomes (with an aqueous lumen) of phospholipids and free cholesterol. LpX is not taken up in atherosclerotic plaques and may reduce the atherogenicity of low-density lipoprotein (LDL) cholesterol by preventing LDL oxidation [34] . Accordingly, the increased serum cholesterol in PBC patients is not associated with increased risk for cardiovascular disease. In contrast, long-lasting PBC is associated with the occurrence of xanthomata and xanthelasma. Hypercholesterolemia in PBC patients is responsive to statin treatment. Long-term treatment with UDCA also reduces serum cholesterol [35] .