Effects of evolocumab on plasma coenzyme Q10 in patients with heterozygous familial hypercholesterolemia

Background and aims: Muscle symptoms are common with statin therapy, and reduced plasma coenzyme Q10 (CoQ10) has been proposed as a potential mechanism. Proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors are widely used in patients with statin intolerance or severe hypercholesterolemia, including familial hypercholesterolemia (FH), but their effects on plasma CoQ10 remain unclear. Methods: We evaluated serum lipid profiles and plasma CoQ10 baseline and 4 weeks after adding evolocumab in patients with heterozygous FH receiving rosuvastatin 10-20 mg/day and ezetimibe 10 mg/day (N = 10; mean age, 52 years; 6 men). Plasma CoQ10 concentrations were determined by high-performance liquid chromatography. Results: Evolocumab markedly reduced LDL cholesterol and lipoprotein(a) [Lp(a)] (121 mg/dL to 64 mg/dL, -50.0%, p < 0.001; 51.8 nmol/L to 39.1 nmol/L, -23.6%, p = 0.002), whereas median CoQ10 levels were unchanged (1028.5 nmol/L to 1002.5 nmol/L, -0.2%, p = 0.33). Changes in CoQ10 did not correlate with reductions in LDL cholesterol or Lp(a). Conclusions: These findings indicate that evolocumab lowers atherogenic lipoproteins without reducing plasma CoQ10, suggesting a potential advantage for patients susceptible to statin-associated muscle symptoms.