Evinacumab

Evinacumab-dgnb is a recombinant human monoclonal antibody that binds to and inhibits ANGPTL3. ANGPTL3 is a member of the angiopoietin-like protein family that is expressed primarily in the liver and plays a role in the regulation of lipid metabolism by inhibiting lipoprotein lipase (LPL) and endothelial lipase (EL).

• EVKEEZA is indicated as an adjunct to diet and exercise and other low-density lipoprotein-cholesterol (LDL-C) lowering therapies to reduce LDL-C in adults and pediatric patients, aged 1 year and older, with homozygous familial hypercholesterolemia (HoFH). EVKEEZA is an angiopoietin-like 3 (ANGPTL3) inhibitor indicated as an adjunct to diet and exercise and other low-density lipoprotein-cholesterol (LDL-C) lowering therapies to reduce LDL-C in adults and pediatric patients, aged 1 year and older, with homozygous familial hypercholesterolemia (HoFH).ICD-10: E78.00

• EVKEEZA is contraindicated in patients with a history of serious hypersensitivity reaction to evinacumab-dgnb or to any of the excipients in EVKEEZA. Serious hypersensitivity reactions, including anaphylaxis, have occurred [see Warnings and Precautions (5.1) ].contraindicated

The recommended dosage of EVKEEZA is 15 mg/kg administered by intravenous (IV) infusion once monthly (every 4 weeks). ( 2.1 ) See the Full Prescribing Information for preparation instructions for the intravenous infusion. ( 2.2 ) 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. ( 2.3 ) Do not mix other medications with EVKEEZA or administer other medications concomitantly via the same infusion line. ( 2.3 ) The rate of infusion may be slowed, interrupted or discontinued if the patient develops any signs of adverse reactions, including infusion or hypersensitivity reactions. ( 2.3 ) 2.1 Recommended Dosage The recommended dosage of EVKEEZA is 15 mg/kg administered by intravenous (IV) infusion over 60 minutes once monthly (every 4 weeks). If a dosage of EVKEEZA is missed, administer as soon as possible. Thereafter, EVKEEZA should be scheduled monthly from the date of the last dosage. Assess LDL-C when clinically appropriate. The LDL-lowering effect of EVKEEZA may be measured as early as 2 weeks after initiation. 2.2 Preparation Instructions for Intravenous Infusion Calculate the dosage (mg), total volume (mL) of EVKEEZA required, and the number of vials required based on the patient's current body weight. Visually inspect the solution for cloudiness, discoloration, and particulate matter prior to administration. EVKEEZA is a clear to slightly opalescent, colorless to pale-yellow solution.

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. ( 5.1 ) 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. ( 5.2 , 8.1 , 8.3 ) 5.1 Serious Hypersensitivity Reactions Serious hypersensitivity reactions, including anaphylaxis, have occurred with EVKEEZA [see Adverse Reactions (6.1) ] . If signs or symptoms of serious hypersensitivity reactions occur, discontinue EVKEEZA infusion, treat according to the standard-of-care, and monitor until signs and symptoms resolve. EVKEEZA is contraindicated in patients with a history of serious hypersensitivity reaction to evinacumab-dgnb. 5.2 Embryo-Fetal Toxicity Based on the findings in animal reproduction studies, EVKEEZA may cause fetal harm when administered to pregnant patients. Administration of evinacumab-dgnb to rabbits during organogenesis caused increases in fetal malformations at doses below the human exposure. Advise patients who may become pregnant of the risk to a fetus. Consider obtaining a pregnancy test prior to initiating treatment with EVKEEZA.

The following clinically significant adverse reactions are described elsewhere in the labeling: Serious Hypersensitivity Reactions [see Warnings and Precautions (5.1) ] Common adverse reactions (≥5%) were nasopharyngitis, influenza-like illness, dizziness, rhinorrhea, nausea, and fatigue. ( 6.1 ) To report SUSPECTED ADVERSE REACTIONS, contact Regeneron at 1-833-385-3392 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch . 6.1 Clinical Trials Experience Because clinical trials are conducted under widely varying conditions, adverse reaction rates observed in the clinical trials of a drug cannot be directly compared to rates in the clinical trials of another drug and may not reflect the rates observed in practice. Adverse Reactions in Adult and Pediatric Patients (aged 12 to 17 years) with HoFH Safety data are based on pooled results from two randomized, double-blind, placebo-controlled trials that included 81 patients treated with EVKEEZA. The mean age of EVKEEZA-treated patients was 48 years (range: 15 to 75 years); 52% were females; 5% were Hispanic; 82% were White, 7% Asian, 3% Black or African American, and 9% other races. Forty-four (54%) EVKEEZA-treated patients had HoFH. Patients received EVKEEZA as add-on therapy to other lipid-lowering therapies, including maximally tolerated statin, ezetimibe, proprotein convertase subtilisin kexin type 9 (PCSK9) inhibitors, lomitapide, and apheresis. Adverse reactions led to discontinuation of treatment in 1 (2%) patient who received placebo, and 2 (2%) patients treated with EVKEEZA, including 1 case of anaphylaxis.

8.1 Pregnancy Risk Summary Based on data from animal reproduction studies, EVKEEZA may cause fetal harm when administered to pregnant patients. Available human data are insufficient to evaluate for a drug-associated risk of major birth defects, miscarriage or adverse maternal or fetal outcomes. Evinacumab-dgnb is a human IgG4 monoclonal antibody [see Description (11) ], and human IgG is known to cross the placental barrier; therefore, evinacumab-dgnb has the potential to be transmitted from the mother to the developing fetus. Subcutaneous administration of evinacumab-dgnb to pregnant rabbits during the period of organogenesis resulted in fetal malformations (domed head, hydrocephalus, and flexed limbs) at doses below the maximum recommended human dose (MRHD). No adverse embryofetal effects were observed with subcutaneous administration of evinacumab-dgnb to pregnant rats during the period of organogenesis at doses below the MRHD. Measurable evinacumab-dgnb serum concentrations were observed in fetal rabbit and rat sera at birth, indicating that evinacumab-dgnb, like other IgG antibodies, crosses the placental barrier (see Data ). Advise pregnant women of the potential risk to a fetus. The estimated background risk of major birth defects and miscarriage for the indicated population are unknown. In the U.S.

Metabolism

The pharmacokinetic parameters described in this section are presented following administration of evinacumab-dgnb 15 mg/kg intravenously every 4 weeks, unless otherwise specified. Steady-state is reached after 4 doses, and the accumulation ratio is 2.

• Feb 11, 2021BLABLA761181Regeneron Pharmaceuticals

1. 1Nausea3315%
2. 2Diarrhoea2813%
3. 3Hepatic Steatosis2511%
4. 4Fatigue2411%
5. 5International Normalised Ratio Increased209.0%
6. 6Headache146.3%
7. 7Product Dose Omission Issue146.3%
8. 8Off Label Use135.8%
9. 9Pain125.4%
10. 10Dizziness114.9%
11. 11Hospitalisation104.5%
12. 12Drug Ineffective94.0%
13. 13Pain In Extremity94.0%
14. 14Illness83.6%
15. 15Influenza Like Illness83.6%

The 10 most recently updated of 15 ClinicalTrials.gov registrations naming Evinacumab as an intervention. Registration is not evidence of efficacy or safety — reference crosswalk only.

• Study to Assess the Effects of Angiopoietin-like Protein 3 (ANGPTL3) Inhibition in Adult Participants With Diabetic Kidney Disease
  Recruiting · Phase 2 · Interventional · 270 enrolled · Regeneron Pharmaceuticals
  NCT07271186updated 2026-05-20
• Assessing the Impact of Intensification of Lipid Lowering Therapy With Guidelines-based Evinacumab Administration on Coronary Plaque Volumes Measured by Coronary Computed Tomography Angiography (CCTA) in Patients With Homozygous Familial Hypercholesterolemia (HoFH)
  Recruiting · Observational · 52 enrolled · Fondazione SISA (Societa Italiana per lo Studio della Arteriosclerosi)
  NCT07447648updated 2026-05-14
• Compassionate Use of Evinacumab
  Approved for marketing · Expanded access · Regeneron Pharmaceuticals
  NCT06500598updated 2025-10-28
• Evaluate the Long-Term Safety and Efficacy of Evinacumab in Patients With Homozygous Familial Hypercholesterolemia
  Completed · Phase 3 · Interventional · 116 enrolled · Regeneron Pharmaceuticals
  NCT03409744updated 2025-04-08
• Safety and Effectiveness of Evinacumab for the Treatment of Homozygous Familial Hypercholesterolemia
  Completed · Phase 3 · Interventional · 10 enrolled · Daniel Gaudet
  NCT05611528updated 2025-03-25
• Evaluate the Efficacy and Safety of Evinacumab in Pediatric Patients With Homozygous Familial Hypercholesterolemia
  Completed · Phase 3 · Interventional · 20 enrolled · Regeneron Pharmaceuticals
  NCT04233918updated 2024-07-16
• Efficacy and Safety of Evinacumab in Adult Patients With Severe Hypertriglyceridemia for the Prevention of Recurrent Acute Pancreatitis
  Terminated · Phase 2 · Interventional · 21 enrolled · Regeneron Pharmaceuticals
  NCT04863014updated 2024-05-22
• Safety and Efficacy Following Repeat-Dose of Evinacumab (Anti-ANGPTL3) in Patients With Severe Hypertriglyceridemia (sHTG) at Risk for Acute Pancreatitis
  Completed · Phase 2 · Interventional · 52 enrolled · Regeneron Pharmaceuticals
  NCT03452228updated 2023-02-16
• Study of Evinacumab (REGN1500) in Participants With Persistent Hypercholesterolemia
  Completed · Phase 2 · Interventional · 272 enrolled · Regeneron Pharmaceuticals
  NCT03175367updated 2023-02-10
• Efficacy and Safety of Evinacumab in Patients With Homozygous Familial Hypercholesterolemia
  Completed · Phase 3 · Interventional · 65 enrolled · Regeneron Pharmaceuticals
  NCT03399786updated 2021-05-18

Frequently asked questions

How does Evinacumab work?

Evinacumab-dgnb is a recombinant human monoclonal antibody that binds to and inhibits ANGPTL3. ANGPTL3 is a member of the angiopoietin-like protein family that is expressed primarily in the liver and plays a role in the regulation of lipid metabolism by inhibiting lipoprotein lipase (LPL) and endothelial lipase (EL).

What is Evinacumab used for?

According to FDA labeling, Evinacumab carries indications including: EVKEEZA is indicated as an adjunct to diet and exercise and other low-density lipoprotein-cholesterol (LDL-C) lowering therapies to reduce LDL-C in adults and pediatric patients, aged 1 year and older, with homozygous familial hypercholesterolemia (HoFH). EVKEEZA is an angiopoietin-like 3 (ANGPTL3) inhibitor indicated as an adjunct to diet and exercise and other low-density lipoprotein-cholesterol (LDL-C) lowering therapies to reduce LDL-C in adults and pediatric patients, aged 1 year and older, with homozygous familial hypercholesterolemia (HoFH).. This is a reference summary of labeled uses, not medical advice or a treatment recommendation.

What class of drug is Evinacumab?

Evinacumab is classified as Other lipid modifying agents, Angiopoietin-like 3 Inhibitor, Angiopoietin-like 3 Inhibitors, Antibody Interactions.

What are the brand names for Evinacumab?

Evinacumab is marketed under brand names including Evkeeza.

What are the contraindications for Evinacumab?

Evinacumab labeling lists contraindications including: EVKEEZA is contraindicated in patients with a history of serious hypersensitivity reaction to evinacumab-dgnb or to any of the excipients in EVKEEZA. Serious hypersensitivity reactions, including anaphylaxis, have occurred [see Warnings and Precautions (5.1) ].. Always consult the full prescribing information and a clinician.