Familial Hypercholesterolaemia

Management

Fibrates

Fibrates increase lipoprotein lipase activity via PPAR-alpha agonism

Ezetimibe

reduces intestinal absorption of cholesterol

Evolocumab (anti-PCSK9 mAb)

  • NICE recommend use of evolocumab when LDL cholesterol is persistently above 3.5 mmol/l.
  • Dose - 140mg every 2 weeks
  • Mechanism - PCSK9 circulates in the blood, and binds to liver LDL-receptors, causing their degradation. Blocking PCSK9 prevents LDLR degradation, allowing uptake of circulating serum LDL-cholesterol
  • Typically lowers LDL cholesterol by more than 50% in 85% of patients who are treated.
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