Evinacumab-dgnb (EvkeezaTM) is an angiopoietin-like protein 3 (ANGPTL3) inhibitor. ANGPTL3 is expressed primarily in the liver and plays a role in the regulation of lipid metabolism by inhibiting lipoprotein lipase and endothelial lipase. Inhibition of ANGPTL3 leads to a reduction in LDL-C, HDL-C and triglycerides.
Evinacumab-dgnb (Evkeeza) may be considered medically necessary when the following criteria are met:
Table 1
Documentation of Homozygous Familial Hypercholesterolemia (must meet either genetic confirmation or clinical confirmation column) |
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Genetic Confirmation |
Clinical Documentation (ONE from each of the following) |
Two (2) mutant alleles at the LDLR, ApoB, PCSK9, or LDLRAP1 gene locus |
ONE of the following untreated lab values |
Untreated total cholesterol of greater than 500 mg/dL |
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Untreated LDL-C of greater than 400 mg/dL |
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Attestation of ONE of the following |
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Cutaneous or tendon xanthoma before age 10 years |
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Evidence of heterozygous familial hypercholesterolemia in both parents |
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Table 2
Statin Therapy Failure (must meet either statin failure or statin intolerance column) |
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Statin Failure (ONE of the following) |
Statin Intolerant (One of the following) |
17 years of age or younger LDL-C greater than 135 mg/dL, despite use of a maximally tolerated statin |
Statin related rhabdomyolysis or skeletal-related muscle symptoms while receiving at least two (2) separate trials of different statins which resolved upon discontinuation of the statins |
18 years of age or older LDL-C greater than 100 mg/dL, despite use of a maximally tolerated statin |
Creatinine kinase (CK) increase to 10 times upper limit of normal (ULN) during any one (1) course of statin therapy |
Liver function tests (LFTs) increase to 3 times upper limit of normal (ULN) during any one (1) course of statin therapy |
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Hospitalization due to severe statin-related adverse event, such as rhabdomyolysis during any one (1) course of statin therapy |
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Table 3
PCSK9 Failure or Contraindication/Adverse Event (must meet either column) |
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PCSK9 Treatment Failure |
PCSK9 Contraindication/Adverse Event |
13 to 17 years of age LDL-C greater than 135 mg/dL, despite use of a PCSK9 inhibitor (evolocumab) for at least three (3) months |
Contraindication to or adverse event from PCSK9 inhibitor therapy |
18 years of age or older LDL-C greater than 100 mg/dL, despite use of a PCSK9 inhibitor (alirocumab or evolocumab) for at least three (3) months |
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Evinacumab-dgnb (Evkeeza) for any other indication is considered experimental/investigational and therefore, not covered. The safety and/or efficacy cannot be established by review of the available published peer-reviewed literature.
J3590 |
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NOTE: In addition to the above criteria, product specific dosage and/or frequency limits may apply in accordance with the U.S. Food and Drug Administration (FDA)-approved product prescribing information, national compendia, Centers for Medicare and Medicaid Services (CMS) and other peer reviewed resources or evidence-based guidelines. Highmark may deny, in full or in part, reimbursement for utilization that does not fall within the applicable dosage and/or frequency limits.
C9079 |
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Refer to Medical Policy I-142, Proprotein Convertase Subtilisin Kexin 9 (PCSK9) Inhibitors for additional information.
E78.01 |
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This policy is designed to address medical guidelines that are appropriate for the majority of individuals with a particular disease, illness, or condition. Each person's unique clinical or other circumstances may warrant individual consideration, based on review of applicable medical records, as well as other regulatory, contractual and/or legal requirements.
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If you believe that the Claims Administrator/Insurer has failed to provide these services or discriminated in another way on the basis of race, color, national origin, age, disability, or sex, you can file a grievance with: Civil Rights Coordinator, P.O. Box 22492, Pittsburgh, PA 15222, Phone: 1-866-286-8295, TTY: 711, Fax: 412-544-2475, email: CivilRightsCoordinator@highmarkhealth.org. You can file a grievance in person or by mail, fax, or email. If you need help filing a grievance, the Civil Rights Coordinator is available to help you.
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Medical policies do not constitute medical advice, nor are they intended to govern the practice of medicine. They are intended to reflect reimbursement and coverage guidelines. Coverage for services may vary for individual members, based on the terms of the benefit contract.
Discrimination is Against the Law
The Claims Administrator/Insurer complies with applicable Federal civil rights laws and does not discriminate on the basis of race, color, national origin, age, disability, or sex. The Claims Administrator/Insurer does not exclude people or treat them differently because of race, color, national origin, age, disability, or sex. The Claims Administrator/ Insurer:
If you believe that the Claims Administrator/Insurer has failed to provide these services or discriminated in another way on the basis of race, color, national origin, age, disability, or sex, you can file a grievance with: Civil Rights Coordinator, P.O. Box 22492, Pittsburgh, PA 15222, Phone: 1-866-286-8295, TTY: 711, Fax: 412-544-2475, email: CivilRightsCoordinator@highmarkhealth.org. You can file a grievance in person or by mail, fax, or email. If you need help filing a grievance, the Civil Rights Coordinator is available to help you.
You can also file a civil rights complaint with the U.S. Department of Health and Human Services, Office for Civil Rights electronically through the Office for Civil Rights Complaint Portal, available at https://ocrportal.hhs.gov/ocr/portal/lobby.jsf, or by mail or phone at:
U.S. Department of Health and Human Services
200 Independence Avenue, SW
Room 509F, HHH Building
Washington, D.C. 20201
1-800-368-1019, 800-537-7697 (TDD)
Complaint forms are available at http://www.hhs.gov/ocr/office/file/index.html.
