How to Get Evkeeza (Evinacumab-dgnb) Covered by Cigna in Illinois: Complete Prior Authorization and Appeals Guide

Answer Box: Getting Evkeeza Covered by Cigna in Illinois

Eligibility: Patients 5+ with confirmed homozygous familial hypercholesterolemia (HoFH) who've failed maximum statin, ezetimibe, and PCSK9 inhibitor therapy.

Fastest approval path: 1) Gather genetic testing confirming HoFH, recent LDL-C levels ≥70 mg/dL on maximum therapy, and pharmacy records of prior treatment failures. 2) Submit prior authorization through EviCore by Evernorth portal. 3) Request expedited review if medically urgent (72-hour decision vs. standard 5-15 days).

Start today: Contact your prescriber's office to initiate the PA request through Cigna's EviCore portal, ensuring complete HoFH documentation is included.


Table of Contents

  1. Coverage Basics
  2. Prior Authorization Process
  3. Step Therapy Requirements
  4. Timeline and Processing
  5. Common Denial Reasons
  6. Appeals Process in Illinois
  7. Cost and Patient Assistance
  8. Renewal Requirements
  9. Specialty Pharmacy Requirements
  10. Troubleshooting

Coverage Basics

Is Evkeeza Covered by Cigna?

Yes, Cigna covers Evkeeza (evinacumab-dgnb) for homozygous familial hypercholesterolemia, but prior authorization is required for all patients. The medication is typically managed through Express Scripts/Accredo specialty pharmacy and requires documentation of specific clinical criteria.

Which Cigna Plans Cover Evkeeza?

Evkeeza coverage applies to most Cigna commercial plans in Illinois, including employer-sponsored insurance and individual marketplace plans. Coverage criteria remain consistent across plan types, though your specific copay or coinsurance may vary based on your plan design.

Coverage at a Glance

Requirement What It Means Where to Find It
Prior Authorization Required for all patients Cigna PA Policy
Age Requirement 5 years or older FDA labeling
HoFH Diagnosis Genetic testing preferred Clinical criteria accepted
Step Therapy Statin + ezetimibe + PCSK9 inhibitor Cigna formulary requirements
Specialty Pharmacy Express Scripts/Accredo Cigna member services
Appeals Deadline 180 days in Illinois Illinois insurance law

Prior Authorization Process

Who Submits the Request?

Your prescriber's office initiates all prior authorization requests through EviCore by Evernorth, Cigna's pharmacy drug PA portal. Patients cannot submit PA requests directly.

Required Documentation

Your medical team must provide:

  • HoFH confirmation: Genetic testing showing two pathogenic mutations (preferred) or clinical phenotype documentation
  • Recent lipid panel: LDL-C levels within 90 days showing ≥70 mg/dL despite maximum tolerated therapy
  • Prior therapy documentation: Pharmacy records and clinical notes showing trials of:
    • High-intensity statin at maximum tolerated dose
    • Ezetimibe 10mg daily
    • PCSK9 inhibitor (evolocumab or alirocumab) for minimum 8 weeks
  • Patient weight: Required for weight-based dosing calculation (15 mg/kg every 4 weeks)
  • Prescriber credentials: Board certification in relevant specialty

How to Check Status

Contact Cigna member services at 1-800-882-4462 or check through your provider's EviCore portal. Have your member ID and the PA request number ready.


Step Therapy Requirements

Cigna requires documented failure of specific background therapies before approving Evkeeza. This step therapy sequence is mandatory:

Required Prior Treatments

1. Maximally Tolerated Statin

  • High-intensity statin (atorvastatin 80mg or rosuvastatin 40mg equivalent)
  • Documented in pharmacy records with adequate trial period
  • If intolerant, provide documentation of adverse effects

2. Ezetimibe 10mg Daily

  • Must be added to statin therapy unless contraindicated
  • Continued concurrent with Evkeeza if approved

3. PCSK9 Inhibitor Trial

  • Minimum 8 continuous weeks on evolocumab or alirocumab
  • LDL-C levels must remain ≥70 mg/dL despite combination therapy
  • Required for patients 10 years and older

Step Therapy Exceptions

Illinois law allows exceptions when:

  • Previous therapy was ineffective (persistent LDL-C ≥70 mg/dL)
  • Patient experienced adverse reactions or contraindications
  • Patient is stable on current Evkeeza therapy (renewals)
  • Standard therapy would harm the patient's condition

Timeline and Processing

Standard Review Timeline

  • Initial submission: 5-15 business days for complete requests
  • Incomplete submissions: Additional 5-10 days while gathering missing information
  • Expedited review: 72 hours for urgent medical needs

When to Request Expedited Review

Request expedited processing if:

  • Delay would seriously jeopardize your health
  • You're currently on Evkeeza and facing treatment interruption
  • Your condition is rapidly worsening without treatment
Tip: Submit expedited requests only when genuinely urgent. Routine requests processed as "urgent" may face additional scrutiny.

Common Denial Reasons

Denial Reason How to Overturn
Missing HoFH confirmation Submit genetic testing or detailed clinical phenotype documentation
Inadequate prior therapy Provide pharmacy records showing 8+ weeks of PCSK9 inhibitor trial
Recent LDL-C not provided Submit lipid panel within 90 days showing LDL-C ≥70 mg/dL
Age requirement not met Confirm patient is 5+ years old in medical records
Not on background therapy Document continued statin + ezetimibe unless contraindicated

Strengthening Your Initial Request

Include these elements to reduce denial risk:

  • Complete medication history with specific dates and dosages
  • Laboratory trend showing persistent elevated LDL-C despite maximum therapy
  • Specialist's clinical rationale explaining why Evkeeza is medically necessary
  • Documentation of HoFH impact on cardiovascular risk

Appeals Process in Illinois

Internal Appeals

If your initial PA request is denied, you have 180 days to file an internal appeal with Cigna. The appeal must include:

  • Completed Cigna appeal form
  • Original denial letter
  • Additional medical documentation supporting medical necessity
  • Updated clinical information if available

Timeline: Cigna provides written decisions within 30 calendar days for standard appeals, or 72 hours for expedited appeals.

External Review Process

Illinois residents have strong appeal rights. If Cigna denies your internal appeal, you can request external review through an Independent Review Organization (IRO).

Key Illinois Requirements:

  • Must request external review within 30 days of final denial (shorter than many states)
  • IRO decision made within 5 business days of receiving all materials
  • No cost to patients - insurers pay review fees
  • IRO decision is binding on Cigna

Illinois Resources for Help

  • Illinois Department of Insurance: (877) 527-9431 for external review guidance
  • Illinois Attorney General Health Care Helpline: (877) 305-5145 for insurance problems
  • Online external review forms available at Illinois DOI website
Note: Illinois' 30-day external review deadline is stricter than many states. Act promptly after receiving a final appeal denial.

Cost and Patient Assistance

Typical Patient Costs

Evkeeza costs approximately $450,000 annually, making insurance coverage essential. Your out-of-pocket costs depend on your specific plan design:

  • Specialty tier copay: Typically $50-200 per month
  • Coinsurance plans: Usually 20-40% of medication cost
  • Deductible: May apply before coverage begins

Patient Assistance Program

The EVKEEZA Patient Assistance Program provides:

  • Up to $25,000 per calendar year toward out-of-pocket costs
  • Covers deductibles and other qualifying expenses
  • Available for eligible patients with commercial insurance

Contact the program directly through the manufacturer's website for enrollment details.

Illinois-Specific Cost Resources

  • Illinois Department of Healthcare and Family Services: Medicaid coverage information
  • Legal aid organizations: Assistance with complex appeals for low-income patients
  • Citizen Action/Illinois: Consumer advocacy for insurance issues

Renewal Requirements

Authorization Duration

  • Initial approval: 6 months
  • Reauthorization: 12 months for established therapy

Required Renewal Documentation

To maintain coverage during reauthorization:

  • Treatment response: LDL-C levels showing improvement on Evkeeza therapy
  • Continued background therapy: Documentation of ongoing statin treatment (if tolerated)
  • Adherence confirmation: Records showing consistent Evkeeza infusions

Renewal Timeline

Submit reauthorization requests 30 days before your current approval expires to avoid treatment interruption. Your specialist's office should track these deadlines and submit renewal requests automatically.


Specialty Pharmacy Requirements

When Evkeeza is approved, Cigna typically requires use of Express Scripts/Accredo specialty pharmacy. This process involves:

  1. Enrollment: Accredo contacts you to set up services and coordinate infusion scheduling
  2. Site of care: Infusions administered at hospital outpatient facility or infusion center
  3. Coordination: Specialty pharmacy works with your infusion site to ensure medication availability

What to Expect

  • Accredo will contact you within 48-72 hours of PA approval
  • They'll coordinate with your chosen infusion center
  • Monthly medication shipments directly to the infusion facility
  • Patient support services for questions and scheduling

Troubleshooting

Common Issues and Solutions

Portal Access Problems

  • Contact EviCore technical support for provider portal issues
  • Ensure your prescriber's office has current Cigna provider credentials

Missing Forms or Documentation

  • Request specific missing items from Cigna's PA team
  • Submit additional documentation promptly to avoid delays

Communication Delays

  • Follow up within 5 business days if no response received
  • Request expedited review for time-sensitive situations

When to Escalate

Contact Illinois regulators if:

  • Cigna doesn't respond within stated timeframes
  • You believe coverage was wrongfully denied
  • Internal appeals process isn't followed properly

Illinois Department of Insurance: (877) 527-9431 for formal complaints and regulatory intervention.


Clinician Corner: Medical Necessity Letter Checklist

Healthcare providers can strengthen PA requests by including:

Clinical Rationale:

  • HoFH diagnosis with genetic confirmation or clinical phenotype
  • Cardiovascular risk assessment specific to patient
  • Documentation of inadequate response to maximum conventional therapy

Evidence Base:

  • FDA labeling supporting HoFH indication
  • Published efficacy data for ANGPTL3 inhibition
  • Clinical guidelines recommending advanced therapies for refractory HoFH

Treatment Plan:

  • Specific dosing based on patient weight (15 mg/kg every 4 weeks)
  • Monitoring plan for LDL-C response and safety parameters
  • Integration with existing lipid-lowering therapy

Frequently Asked Questions

How long does Cigna PA take in Illinois? Standard review takes 5-15 business days for complete submissions. Expedited review provides decisions within 72 hours for urgent cases.

What if Evkeeza is non-formulary on my plan? Submit a formulary exception request with medical necessity documentation. Cigna must review non-formulary requests using the same clinical criteria.

Can I request an expedited appeal? Yes, if standard timing would jeopardize your health or interrupt current treatment. Provide documentation of medical urgency with your appeal.

Does step therapy apply if I failed treatments outside Illinois? Yes, pharmacy records and clinical documentation from any state satisfy Cigna's step therapy requirements.

What happens if my appeal is denied? Illinois law guarantees external review by an independent physician specialist. Request external review within 30 days of your final appeal denial.


Counterforce Health helps patients and clinicians navigate complex insurance denials by creating targeted, evidence-backed appeals. Our platform analyzes denial letters and plan policies to identify the specific reasons for coverage denials, then drafts point-by-point rebuttals aligned with the insurer's own rules. For medications like Evkeeza, we pull the right clinical evidence - from FDA labeling to peer-reviewed studies - and weave it into appeals with all the required documentation insurers expect.


Disclaimer: This information is for educational purposes and should not replace professional medical or legal advice. Insurance coverage policies change frequently. Always verify current requirements with Cigna directly and consult with your healthcare provider about treatment decisions.

Sources & Further Reading

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