Evkeeza (evinacumab-dgnb) is an angiopoietin-like 3 inhibitor indicated as an adjunct to diet and exercise and other low density lipoprotein-cholesterol lowering therapies to reduce LDL-C in adults and pediatric patients with homozygous familial hypercholesterolemia. The drug was originally approved in February of 2021 for patients 12 and older, but in 2023 it was expanded to include patients five and older, and then expanded again, late last year, to include patients one year old, and older.
Read on to learn more about Evkeeza, including its indications timeline, dosage and administration, related guideline mentions, and more.
Medication Overview:
- Brand Name: Evkeeza
- Generic Name: evinacumab-dgnb
- Treatment for: Homozygous familial hypercholesterolaemia
- Manufacturer(s): Regeneron
- Initial FDA Approval: February 2021
Evkeeza Indications Timeline Table
| Indicated Condition | Indication | Age | Date Approved |
|---|---|---|---|
| Homozygous familial hypercholesterolaemia | Approved as an adjunct to diet and exercise and other lipid-lowering therapies for the treatment of children from age 1 to less than 5 years old with homozygous familial hypercholesterolemia (HoFH). | 1 to 5 years old | September 2025 |
| Homozygous familial hypercholesterolaemia | Approved as an adjunct to other lipid-lowering therapies to treat children aged 5 to 11 with homozygous familial hypercholesterolemia (HoFH). | 5 to 11 years old | March 2023 |
| Homozygous familial hypercholesterolaemia | Approved as an adjunct to other low-density lipoprotein cholesterol (LDL-C) lowering therapies to treat adult and pediatric patients aged 12 years and older with homozygous familial hypercholesterolemia (HoFH) | 12 years and older | February 2021 |
Warnings and Precautions:
Serious Hypersensitivity Reactions: Have occurred with Evkeeza in clinical trials. If a serious hypersensitivity reaction occurs, discontinue Evkeeza, treat according to standard-of-care and monitor until signs and symptoms resolve.
Embryo-Fetal Toxicity: Evkeeza may cause fetal harm based on animal studies. Advise patients who may become pregnant of the risk to a fetus. Consider obtaining a pregnancy test prior to initiating treatment with Evkeeza. Advise patients who may become pregnant to use contraception during treatment and for at least 5 months following the last dosage.
Dosage and Administration:
The recommended dosage of Evkeeza is 15 mg/kg administered by intravenous (IV) infusion once monthly (every 4 weeks).
See the Full Prescribing Information for preparation instructions for the intravenous infusion.
Administer the diluted solution via IV infusion over 60 minutes through an IV line containing a sterile, in-line or add-on, 0.2-micron to 5-micron filter.
Do not mix other medications with Evkeeza or administer other medications concomitantly via the same infusion line.
Contraindications:
History of serious hypersensitivity reactions to evinacumab-dgnb or to any of the excipients in Evkeeza.
Adverse Reactions:
Common adverse reactions ("5%) were nasopharyngitis, influenza-like illness, dizziness, rhinorrhea, nausea, and fatigue.
Examples of Evinacumab in Guidelines
- European Society of Cardiology / European Atherosclerosis Society, November 2025
- “In patients with homozygous FH, in whom statins and PCSK9 inhibitors have little efficacy and may not suffice to adequately lower LDL-C levels,49 evinacumab, an mAb against ANGPTL3 (angiopoietin-like 3), has shown potential benefits with a reduction of LDL-C close to 50%.”
Homozygous Familial Hypercholesterolaemia - New Treatments and Clinical Guidance
- European Atherosclerosis Society, July 2023
- “Data with these newer agents are, however, limited in children with HoFH. To address this, the panel strongly supports creation of a fast-tracked regulatory pathway for approval of novel treatments in young children with HoFH; e.g. evinacumab was recently approved for children aged 5–11 years by the US Food and Drug Administration.”
Please note: This article is current as of January 16, 2026. Consult our clinical guidelines library or drug information tool to ensure you always have the most up-to-date information.
Sign up for alerts to keep up with the latest clinical guidelines and guideline updates.
Copyright © 2026 Guideline Central, all rights reserved.