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.
Preferred Products
Proprotein convertase substilisin kexin 9 (PCSK9) inhibitors are the preferred products required for members initiating new therapy for homozygous familial hypercholesterolemia. The non-preferred product [evinacumab-dgnb (Evkeeza)] will be considered when the member has a documented therapy failure after an adequate therapeutic trial of a preferred product, the preferred product has not been tolerated or is contraindicated or the individual does not meet minimum age requirements based on FDA approval for the preferred products.
Adequate therapeutic trial is defined as three (3) months following the injection series at Food and Drug Administration (FDA) or compendia based therapeutic doses of preferred product.
New therapy is defined as no previous utilization within the last 365 calendar days.
Evinacumab-dgnb (Evkeeza) may be considered medically necessary when the following criteria are met:
Reauthorization Criteria
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 |
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) |
7 to 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 |
Table 3
PCSK9 Failure or Contraindication/Adverse Event (must meet either column) |
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PCSK9 Treatment Failure |
PCSK9 Contraindication/Adverse Event |
10 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 not medically necessary.
J1305 |
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Evinacumab-dgnb (Evkeeza) may be considered medically necessary for individuals 18 years of age and older when applicable clinical criteria for individual medication policies are met and when administered in a physician’s office not affiliated with a hospital, specialized infusion centers not affiliated with a hospital or in the home.
Outpatient facility (Outpatient Hospital IV Infusion Department or Hospital-based Outpatient Clinical Level of Care) administration may be considered medically necessary if ANY of the following criteria are present to indicate the member is medically unstable for infusions in other than an outpatient facility setting:
Home health services may be considered medically necessary when utilized for the administration of home infusion therapy and when provided by licensed eligible provider. Each case will be addressed on an individual basis.
The medications identified in this policy will be considered not medically necessary if administered in an unapproved hospital outpatient setting when an approved site of care is a viable option for treatment.
J1305 |
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Refer to Medical Policy I-151, Site of Care, for additional information.
E78.01 |
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Evidence based guidelines support the administration of this drug in alternative sites of care such as the home, office or outpatient ambulatory infusion centers. Administration of infusible drugs at alternate sites of care is based upon the professional judgment of the provider, and taken into account the clinical appropriateness for each individual patient.
Evidence-based guidelines support the administration of injectable medications in alternative sites of care such as the non-hospital physician’s office, non-hospital infusion center or in the home. Administration of the injectable medications subject to this policy at alternate sites of care is based upon the professional judgment of the provider, and takes into account the clinical appropriateness for each individual member. Requests for administration of any dose of the drugs listed in this policy received from a hospital-based facility, physician’s office or specialized infusion center will be assessed for meeting the policy exception criteria based on the clinical documentation provided by the requesting practitioner. is typically an outpatient procedure which is only eligible for coverage as an inpatient procedure in special circumstances, including, but not limited to, the presence of a co-morbid condition that would require monitoring in a more controlled environment such as the inpatient setting.
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.
Medical policies do not constitute medical advice, nor are they intended to govern the practice of medicine. They are intended to reflect Highmark's reimbursement and coverage guidelines. Coverage for services may vary for individual members, based on the terms of the benefit contract.
Highmark retains the right to review and update its medical policy guidelines at its sole discretion. These guidelines are the proprietary information of Highmark. Any sale, copying or dissemination of the medical policies is prohibited; however, limited copying of medical policies is permitted for individual use.
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 need these services, contact the Civil Rights Coordinator.
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.
This information is issued by Highmark Blue Shield on behalf of its affiliated Blue companies, which are independent licensees of the Blue Cross Blue Shield Association. Highmark Inc. d/b/a Highmark Blue Shield and certain of its affiliated Blue companies serve Blue Shield members in the 21 counties of central Pennsylvania. As a partner in joint operating agreements, Highmark Blue Shield also provides services in conjunction with a separate health plan in southeastern Pennsylvania. Highmark Inc. or certain of its affiliated Blue companies also serve Blue Cross Blue Shield members in 29 counties in western Pennsylvania, 13 counties in northeastern Pennsylvania, the state of West Virginia plus Washington County, Ohio, the state of Delaware[ and [8] counties in western New York and Blue Shield members in [13] counties in northeastern New York]. All references to Highmark in this document are references to Highmark Inc. d/b/a Highmark Blue Shield and/or to one or more of its affiliated Blue companies.
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.