Pharmacy Prior Authorization Criteria

Evkeeza (evinacumab-dgnb)

Indications for Prior Authorization

Evkeeza (evinacumab-dgnb)

• For diagnosis of Homozygous Familial Hypercholesterolemia (HoFH)
  Indicated as an adjunct to other low-density lipoprotein-cholesterol (LDL-C) lowering therapies for the treatment of adult and pediatric patients, aged 5 years and older, with homozygous familial hypercholesterolemia (HoFH).

• For diagnosis of Limitations of Use
  The safety and effectiveness of Evkeeza have not been established in patients with other causes of hypercholesterolemia, including those with heterozygous familial hypercholesterolemia (HeFH). The effects of Evkeeza on cardiovascular morbidity and mortality have not been determined.

Criteria

Evkeeza

Prior Authorization (Initial Authorization)

Length of Approval: 6 Months [A]
For diagnosis of Homozygous Familial Hypercholesterolemia [HoFH]

• Diagnosis of homozygous familial hypercholesterolemia (HoFH) as confirmed by one of the following: [2]
• Genetic confirmation of two mutant alleles at the LDLR, APOB, PCSK9, or LDLRAP1 gene locus
OR
• Both of the following:
• Untreated LDL-C greater than 400 mg/dL
AND
• One of the following:
• Xanthoma before 10 years of age
• Evidence of heterozygous familial hypercholesterolemia in both parents
AND
• Patient is 5 years of age or older
AND
• One of the following:
• Patient is currently treated with maximally tolerated statin therapy
• Patient is statin intolerant as evidenced by an inability to tolerate at least two statins, with at least one started at the lowest starting daily dose, due to intolerable symptoms or clinically significant biomarker changes of liver function or muscle function (e.g., creatine kinase)
• Patient has a contraindication to all statins
AND
• One of the following:
• Patient has been receiving ezetimibe (Zetia) therapy as adjunct to maximally tolerated statin therapy
• Patient has a history of intolerance or contraindication to ezetimibe
• Patient is less than 10 years of age
AND
• One of the following:
• Patient has been treated with PCSK9 therapy or did not respond to PCSK9 therapy
• Physician attests that the patient is known to have two LDL-receptor negative alleles (little to no residual function) and therefore would not respond to PCSK9 therapy
• Patient has a history of intolerance or contraindication to PCSK9 therapy
• Patient is less than 10 years of age
AND
• Patient will continue other traditional lipid-lowering therapies (e.g., maximally tolerated statins, ezetimibe) in combination with Evkeeza
AND
• Dose will not exceed 15 mg/kg of bodyweight infused once every 4 weeks
AND
• Prescribed by or in consultation with one of the following:
• Cardiologist
• Endocrinologist
• Hepatologist

Evkeeza

Prior Authorization (Reauthorization)

Length of Approval: 12 Month(s)
For diagnosis of Homozygous Familial Hypercholesterolemia [HoFH]

• Patient demonstrates positive clinical response to as evidenced by LDL-C reduction from baseline
AND
• Patient will continue other traditional lipid-lowering therapies (e.g., maximally tolerated statins, ezetimibe) in combination with Evkeeza
AND
• Dose will not exceed 15 mg/kg of bodyweight infused once every 4 weeks
AND
• Prescribed by or in consultation with one of the following:
• Cardiologist
• Endocrinologist
• Hepatologist

P & T Revisions

2024-05-03, 2023-08-22, 2023-03-27, 2022-04-01, 2021-04-13

References

1. Evkeeza Prescribing Information. Regeneron Pharmaceuticals, Inc. Tarrytown, NY. March 2023.
2. Cuchel M, Raal FJ, Hegele RA, Al-Rasadi K, Arca M, Averna M, Bruckert E, Freiberger T, Gaudet D, Harada-Shiba M, Hudgins LC, Kayikcioglu M, Masana L, Parhofer KG, Roeters van Lennep JE, Santos RD, Stroes ESG, Watts GF, Wiegman A, Stock JK, Tokgözoğlu LS, Catapano AL, Ray KK. 2023 Update on European Atherosclerosis Society Consensus Statement on Homozygous Familial Hypercholesterolaemia: new treatments and clinical guidance. Eur Heart J. 2023 Jul 1;44(25):2277-2291. doi:10.1093/eurheartj/ehad197. PMID: 37130090; PMCID: PMC10314327.
3. Grundy SM, Stone NJ, Bailey AL, et al. 2018 AHA/ACC/AACVPR/AAPA/ABC/ACPM/ADA/AGS/APhA/ASPC/NLA/PCNA Guideline on the Management of Blood Cholesterol: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2019; 73:e285-e350.

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End Notes

1. Per the 2018 ACC/AHA national treatment guidelines, adherence, response to therapy, and adverse effects should be monitored within 4 -12 weeks following LDL-C lowering medication initiation or dose adjustment, repeated every 3 to 12 months as needed. Additionally, in the Evkeeza pivotal trial the primary outcome of change in LDL-C was evaluated at 24 weeks. [3]
2. In patients treated with statins, it is recommended to measure creatine kinase levels in individuals with severe statin-associated muscle symptoms. [3]

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Revision History

• 2024-05-03: Annual Review - Criteria update
• 2023-08-22: Program update to standard reauthorization language. No changes to clinical intent.
• 2023-03-27: Annual review - submission of medical records removed. Age criteria updated to reflect label update
• 2022-04-01: Annual Review - Removal of "as documented in medical records" from verification of labeled contraindication to all statins Reauth updated to include reduction of LDL-C "from baseline" while on therapy
• 2021-04-13: New Program

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