Lipoprotein apheresis | PCSK9 inhibitors

Lipoprotein apheresis

  • Lipoprotein Apheresis (LA) is an extracorporeal treatment that removes ApoB-containing lipoproteins from the circulation and is usually performed at a transfusion medicine or haemodialysis centre.
  • For patients who require LA, the earlier they are started the better the prognosis. In HoFH patients ideally by age 5 and not later than 8 years.
  • Expected LDL cholesterol reductions:
    • acute – LDL cholesterol level reduction of 55 – 70% can be achieved by a single treatment
    • long-term treatment – weekly or fortnightly can achieve close to normal LDL cholesterol values.
  • Statin and other drug therapies (e.g. Ezetimibe, Niacin) should be continued during LA.
  • Concomitant therapy with an ACE inhibitor is contraindicated owing to the risk of hypotension.
  • The removal of LDL in this high risk category improves:
    • CHD outcomes – e.g. angina pectoris
    • progression of atherosclerosis and aortic fibrosis
    • endothelial function and markers of inflammation.
  • Indications:
    • HoFH patients
    • compound HeFH patients
    • severe HeFH patients with progressive CHD who are refractory or intolerant to maximal cholesterol lowering medication
    • during pregnancy where patients have severely elevated LDL cholesterol levels as in HoFH patients with established CHD.

PCSK9 inhibitors

  • Optimal reduction in plasma levels of LDL cholesterol is not achievable in many FH patients. 
  • Substantial reduction in patient LDL cholesterol has been achieved through the development of highly innovative treatments, such as PCSK9 monoclonal antibodies, used in conjunction with standard therapy.
  • Monoclonal antibodies prevent the binding of PCSK9 to the LDL receptor, thereby preventing degradation of the LDL receptor and increasing the availability of LDL receptors on the cell surface to clear LDL from the circulating blood.
  • PCSK9 inhibitors have been shown to reduce LDL cholesterol levels by up 70%, can be given with statin therapy and are administered by subcutaneous injection fortnightly or monthly.
  • Studies have shown no serious side effects and those displayed are injection site related.