CurrentCignaPolicy N/A

Hereditary Angioedema - Berinert and Cinryze Drug Quantity Management Policy

Defines drug quantity limits, approval criteria, and allowable overrides for Berinert and Cinryze for treatment and prophylaxis of hereditary angioedema for Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyBerinert and Cinryze drug quantity management for hereditary angioedema

Policy CodePolicy N/A

Change TypeRevised quantity override limits and approval duration clarification

Effective DateN/A

Next Review DateN/A

Key ActionRequest prior authorization for quantities exceeding the Drug Quantity Management limits and include documentation of patient weight, age, indication, and prior amounts dispensed.

SourceLink

POLICY UPDATE CHANGES

Overrides for additional vials/kits were increased: for patients ≤100 kg, overrides updated to 16 additional vials/kits (from 4 retail /12 home delivery); for patients >100 kg, overrides updated to 32 additional vials/kits (from 8 retail /24 home delivery).

The policy statement was updated to note that 'one-time' approvals are provided for 30 days in duration.

32Retail max per 28 days

96Home delivery max per 84 days

16Prophylaxis component (≤100 kg)

20/kg

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

Coverage Criteria and Drug Quantity Limits

Drug Quantity Limits and Overrides

Covered as medically necessary when the Drug Quantity Management limits or specified override criteria are met.

Standard quantity limits: Approve up to 32 kits/vials per 28 days at retail or 96 kits/vials per 84 days for home delivery (500-unit kit/vial). This quantity provides prophylaxis at 1,000 units twice weekly (16 kits/vials per 28 days) plus four acute treatment doses (16 kits/vials) for patients up to 100 kg.32/96

Standard limits apply unless an override criterion below is met.

Weight-based increased allowance: If the patient weighs >100 kg, approve 48 kits/vials per 28 days at retail or 144 kits/vials per 84 days at home delivery.>100 kg

Supports increased allowance for patients >100 kg.

Higher prophylaxis dose for ≥12 years (≤100 kg): If the patient is ≥12 years and ≤100 kg and requires a dose >1,000 units twice weekly for long-term prophylaxis, approve 56 kits/vials per 28 days at retail or 168 kits/vials per 84 days at home delivery.>=12 years, <=100 kg, dose >1000 units twice weekly

Approval for selected patients ≥12 years with inadequate response to standard prophylaxis.

Higher prophylaxis dose for ≥12 years (>100 kg): If the patient is ≥12 years and >100 kg and requires a dose >1,000 units twice weekly for long-term prophylaxis, approve 72 kits/vials per 28 days at retail or 216 kits/vials per 84 days at home delivery.>=12 years, >100 kg, dose >1000 units twice weekly

Approval for selected patients ≥12 years with inadequate response to standard prophylaxis.

One-time override for additional acute/short-term prophylaxis doses (<=100 kg): If the patient weighs ≤100 kg and requires additional doses to treat a subsequent HAE attack or short-term (procedural) prophylaxis, approve a one-time override not to exceed 16 additional kits/vials. At retail, the approval quantity = kits/vials received in past 28 days + 16. At home delivery, the approval quantity = kits/vials received in past 84 days + 16. ONE override may be approved once every 30 days.<=100 kg, one-time

One-time approvals are provided for 30 days.

One-time override for additional acute/short-term prophylaxis doses (>100 kg): If the patient weighs >100 kg and requires additional doses to treat a subsequent HAE attack or short-term (procedural) prophylaxis, approve a one-time override not to exceed 32 additional kits/vials. At retail, the approval quantity = kits/vials received in past 28 days + 32. At home delivery, the approval quantity = kits/vials received in past 84 days + 32. ONE override may be approved once every 30 days.>100 kg, one-time

One-time approvals are provided for 30 days.

Requests for Berinert (500‑unit kit) or Cinryze (500‑unit vial) that exceed the point‑of‑service Drug Quantity Management limits are eligible for coverage only when they meet the program’s specified quantity limit criteria or the defined override rules. The standard maximums are 32 kits/vials per 28 days (retail) or 96 kits/vials per 84 days (home delivery), which provide routine prophylaxis at 1,000 units twice weekly plus allowance for acute treatment doses; approvals beyond these amounts require review under the policy’s criteria for increased allowances or one‑time overrides.

Any request that does not meet the Drug Quantity Management limits and does not satisfy one of the policy’s explicit override or increased‑allowance criteria is considered not medically necessary and will be denied. Exceptions must align with the listed criteria (for example, weight‑based increases or approved one‑time overrides) to be approved.

Coding, Dosing, and Units

Covered HCPCS CodesHCPCSCovered

J0598

C1 esterase inhibitor (human)

inv-05: Acute treatment dose — key value

Recommended acute treatment dose20 units/kg

Per-attack maximum units (≤100 kg)Up to 2,000 units (equates to 4 × 500‑unit vials/kits)

Vials/kits required per acute attack (≤100 kg)4 (500‑unit kits/vials)

inv-06: Standard prophylaxis dose (adult 6ge;12 years) — key value

Standard prophylaxis dose (≥12 years)1,000 units IV once every 3–4 days

Escalation for inadequate responseMay increase up to 2,500 units (not to exceed 100 units/kg) once every 3–4 days

Prior Authorization, Documentation, and Operational Rules

Denial Risk

Drug Quantity Management and Coverage Determination

This policy is subject to Cigna's Drug Quantity Management rules. If the Drug Quantity Management rule is not met at the point of sale, coverage will be determined by the clinical Criteria in this policy and may be denied if requirements are not met. All approvals are provided for 1 year unless a one-time approval is granted (one-time approvals = 30 days).

All approvals are for 1 year; one-time approvals = 30 days.

Prior Authorization

Quantity limits and authorization

Drug Quantity Management limits apply to Berinert and Cinryze. Point-of-service quantity limits: Retail maximum per 28 days = 32 kits/vials; Home Delivery maximum per 84 days = 96 kits/vials. These limits provide sufficient quantity for prophylaxis at 1,000 units twice weekly plus treatment of up to four HAE attacks for patients up to 100 kg. Exceptions to these limits require documentation and are handled per the Criteria below.

Definitions and Units

inv-12: Unit — defines C1-INH unit

Definition of UnitPotency expressed in standard units of C1‑INH equal to the mean C1‑INH quantity in 1 mL of normal human plasma.

Unit basis for dosingBoth Berinert and Cinryze potencies are expressed in C1‑INH units

Packaging referenceAvailable as 500‑unit vials/kits (units refer to C1‑INH potency per vial/kit)

Background

Berinert and Cinryze are plasma‑derived C1 esterase inhibitor (C1‑INH) replacement therapies used in hereditary angioedema (HAE). Berinert is supplied as a 500‑unit kit and is indicated for treatment of acute HAE attacks; Cinryze is supplied as 500‑unit vials (including a co‑pack with diluent) and is indicated for routine prophylaxis. Dosing differs by indication: acute treatment is typically 20 units/kg, and prophylaxis is commonly 1,000 units IV every 3–4 days with possible escalation up to 2,500 units or not to exceed 100 units/kg based on clinical response.