ANGPTL3 inhibitor monoclonal antibody Pregnancy: May cause fetal harm based on animal reproduction studies (fetal malformations in rabbits at doses below the maximum recommended human dose); human data insufficient. As a human IgG4 monoclonal antibody it crosses the placenta. Consider a pregnancy test before initiating; advise patients who may become pregnant to use contraception during treatment and for at least 5 months after the last dose.

Evinacumab

Brand names: Evkeeza

Evinacumab is a monoclonal antibody used as an adjunct to other lipid-lowering therapies in homozygous familial hypercholesterolaemia.

Auto-extracted from the source labelling — not yet independently clinician-verified. These values were distilled from the UK SPC (or the US label where noted) but have not had a clinician sign-off. Confirm against the current SmPC before prescribing.

Adult dose

Dose: 15 mg/kg

Route: Intravenous infusion over 60 minutes, through an IV line containing a sterile in-line or add-on 0.2-micron to 5-micron filter

Frequency: Once monthly (every 4 weeks)

Max: Not stated in the source label

Product EVKEEZA (US labelling). Dilute the required volume in 0.9% sodium chloride injection or 5% dextrose injection; final concentration of the diluted solution should be between 0.5 mg/mL and 20 mg/mL depending on current body weight. Maximum diluent volume by patient weight: 3 kg to less than 26 kg = 5 mL/kg; 26 kg to less than 45 kg = 150 mL; 45 kg and greater = 250 mL. Do not shake. Administer immediately after preparation; if not, store refrigerated 2-8 degrees C for no more than 24 hours from preparation, or at room temperature up to 25 degrees C for no more than 6 hours from preparation to end of infusion; do not freeze. Do not mix other medicines with EVKEEZA or give other medicines concomitantly via the same infusion line. The rate of infusion may be slowed, interrupted or discontinued if any signs of adverse reactions occur, including infusion or hypersensitivity reactions. Missed dose: administer as soon as possible, then schedule monthly from the date of the last dose. Can be administered without regard to the timing of lipoprotein apheresis. Assess LDL-C when clinically appropriate — the LDL-lowering effect may be measured as early as 2 weeks after initiation.

Paediatric dose

Dose: 15 mg/kg

Route: Intravenous infusion over 60 minutes through a 0.2-micron to 5-micron in-line or add-on filter

Frequency: Once monthly (every 4 weeks)

Max: Not stated in the source label

The label states a single recommended dosage of 15 mg/kg; safety and effectiveness as an adjunct to other LDL-C-lowering therapies for HoFH have been established in paediatric patients aged 1 year and older, and have NOT been established in patients younger than 1 year. Maximum diluent volume by weight: 3 kg to less than 26 kg = 5 mL/kg; 26 kg to less than 45 kg = 150 mL; 45 kg and greater = 250 mL. Safety profile in patients aged 1 to 11 years was similar to adults, with the additional adverse reaction of fatigue in patients aged 5 to 11 years. US labelling — clinician to verify against the UK SPC and a children's formulary before use.

Dose auto-extracted from US FDA prescribing information (openFDA / DailyMed) — cross-check; US labelling may differ from UK — not yet clinician-verified. Always confirm against the product SmPC and your local formulary before prescribing.

Paediatric weight-based calculator

Patient weight (kg)

Verify in a children's formulary

Contraindications

History of a serious hypersensitivity reaction to evinacumab-dgnb or to any of the excipients (serious hypersensitivity reactions, including anaphylaxis, have occurred)

Side effects

Nasopharyngitis
Influenza-like illness
Dizziness
Rhinorrhoea
Nausea
Fatigue

Clinical monograph

How it works

It inhibits angiopoietin-like protein 3 (ANGPTL3), enhancing lipoprotein and endothelial lipase activity to lower LDL-cholesterol through a pathway largely independent of the LDL receptor.

Prescribing in practice

Serious hypersensitivity reactions, including anaphylaxis, have been reported, so it should be given where such reactions can be managed and the infusion stopped if they occur.
It is indicated specifically as an adjunct to diet and other LDL-lowering treatments in homozygous familial hypercholesterolaemia, not for general hypercholesterolaemia.
Effective contraception is advised because of potential foetal harm, and treatment should be initiated and supervised by a specialist.

Monitoring

Monitor LDL-cholesterol response and observe for infusion-related and hypersensitivity reactions during and after administration.

Counselling the patient

This is given as a hospital infusion under specialist supervision.
Report rash, swelling, breathlessness or other reaction during or after the infusion.
Use reliable contraception and discuss pregnancy plans with your specialist.

Evidence & guidelines

The ELIPSE HoFH trial demonstrated substantial LDL-cholesterol reduction with evinacumab in homozygous familial hypercholesterolaemia, supporting its specialist adjunctive role.

Reference: NICE TA814; SmPC; Drug verified in RxNorm (NLM); confirm dosing against the manufacturer SPC (eMC). Verify against your local formulary and current prescribing references before prescribing. The structured dose values shown have been reviewed by a clinician. Monograph status: clinician-reviewed (2026-07-04).

Curated clinical cross-links plus same-class fallbacks.

Calculators

Pathways