Currentmedical mutual - ohioPolicy SD, 1 = 201020-MRx (10-22)

Berinert (C1 esterase inhibitor [human]) injection prior approval

Prior authorization and coverage criteria for intravenous Berinert (J0597) for treatment of acute hereditary angioedema (HAE) attacks, including site-of-care and medication sourcing provisions, affecting providers who prescribe or administer Berinert under the Medical Mutual - Ohio medical benefit.

Policy Summary

Payermedical mutual - ohio

PolicyBerinert (C1 esterase inhibitor [human]) injection prior approval

Policy CodePolicy SD, 1 = 201020-MRx (10-22)

Change TypeNo material change

Effective DateApr 21, 2016

Next Review DateAug 21, 2025

Key ActionObtain prior authorization for HCPCS J0597; initial approval is valid for 12 weeks and may be renewed every 12 weeks with review of on-hand medication.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

J0597Primary HCPCS code governed

12 weeksInitial prior authorization validity

1000Maximum billable units per 28 days

D84.1Covered diagnosis code

20 IU/kgDose for acute HAE attack

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Coverage Criteria for Berinert (C1 esterase inhibitor [human])

Initial Approval Criteria

Covered when ALL of the following are met

Universal requirements: Must be prescribed by or in consultation with a specialist in allergy, immunology, hematology, pulmonology, or medical genetics; will not be used in combination with another agent indicated for the treatment of acute HAE attacks (e.g., icatibant, ecallantide, recombinant C1-INH) except when reviewed case-by-case; and confirmation the patient is avoiding estrogen-containing oral contraceptives or hormone replacement therapy, ACE inhibitors or ARBs, DPP-IV inhibitors, and neprilysin inhibitors.

Indication-specific: Acute HAE attack: Patient has a history of moderate to severe cutaneous attacks (without urticaria) OR abdominal attacks OR mild to severe airway swelling (debilitating cutaneous/GI symptoms or laryngeal/pharyngeal/tongue swelling).

Prior therapy / special populations: One of: trial of the preferred product Ruconest (documentation required); OR history of at least one laryngeal attack previously treated with Berinert per prescriber; OR allergy to rabbits or rabbit-derived products; OR patient is less than 13 years of age.

Laboratory confirmation: Patient has one of the HAE subtypes confirmed by repeat blood testing: HAE I (low C1-INH antigenic level, low C4, low C1-INH functional level) OR HAE II (normal/elevated C1-INH antigenic level with low C4 and low C1-INH functional level) OR HAE with normal C1-INH (normal/near-normal C1-INH, C4, and function with repeat testing during an attack excluding HAE I/II, plus either a known HAE-causing mutation or family history and lack of response to high-dose antihistamines).

Renewal Criteria

Renewal covered when ALL of the following are met

Renewal general: Patient continues to meet the universal and indication-specific criteria; significant improvement in severity and duration of attacks has been achieved and sustained; and there is absence of unacceptable toxicity from the drug (e.g., severe hypersensitivity, serious thromboembolic events).

Supply consideration: Authorization will account for the cumulative amount of medication the patient has on-hand and will provide a quantity sufficient to treat up to 4 acute attacks per 4 weeks for the duration of the authorization.

Prior therapy / special populations (renewal): One of: trial of the preferred product Ruconest (documentation required); OR history of at least one laryngeal attack treated with Berinert as per prescriber; OR allergy to rabbits or rabbit-derived products; OR patient is less than 13 years of age.

Use of Berinert in combination with another agent indicated for treatment of acute hereditary angioedema attacks (for example, icatibant [Firazyr], ecallantide [Kalbitor], or recombinant C1‑INH [Ruconest]) is not allowed except in circumstances that will be reviewed on a case‑by‑case basis. Requests for combination or duplicate therapy may be considered appropriate only when documented justification is provided and the request is reviewed.

The Company may deny payment or require additional documentation when the drug or related services are determined to be not medically necessary, investigational/experimental, outside the member’s benefits, or part of an inappropriate or excessive billing pattern. Providers must make supporting records (including patient records, test results, and provider credentials) available upon request; failure to substantiate medical necessity may result in denial and providers may be required to write off charges denied as not medically necessary.

Coding and Dosing Information

Covered HCPCS CodesHCPCSCovered

J0597

Injection, C-1 esterase inhibitor (human), berinert, 10 units; 1 billable unit = 10 units

NDCNDC

63833-0825-xx	Berinert 500 IU single-dose carton kit
63833-0835-xx	Berinert 500 IU single-dose vial

Covered Diagnosis CodesICD-10Covered

D84.1

Defects in the complement system

Maximum units per 28 days

Maximum units (28-day period)1000 billable units per 28 days.

Unit definitionHCPCS unit: 1 billable unit = 10 units of Berinert (see HCPCS J0597).

Supply reviewAuthorization will account for cumulative on‑hand medication to ensure limits are not exceeded within the 28‑day period.

Acute attack dosing

Acute attack doseAdminister 20 international units (IU) per kg body weight by intravenous injection.

RouteIntravenous (IV) administration.

Provider Requirements and Authorization

Prior Authorization

Prior Authorization Required

Prior authorization is required for HCPCS J0597. Initial authorization will be provided for 12 weeks and may be renewed every 12 weeks thereafter. When authorizing, the cumulative amount of HAE medication(s) the patient has on-hand will be considered; the authorization will provide a sufficient quantity to treat up to 4 acute attacks per 4 weeks for the duration of the authorization.

Affected code: HCPCS J0597 (Berinert)
Initial authorization: 12 weeks
Renewal authorization: may be renewed every 12 weeks
Quantity consideration: enough to treat up to 4 acute attacks per 4 weeks for the authorization period

Step Therapy

Preferred Product Trial / Alternatives

Requests should document trial of the preferred product Ruconest when required by the clinical criteria. Alternative justifications that may substitute for a Ruconest trial include documentation of a history of at least one laryngeal attack previously treated with Berinert (per the prescriber), a documented allergy to rabbits or rabbit-derived products, or the patient being less than 13 years of age. Provide clear documentation of the trial, intolerance, allergy, or prior treatment as applicable.

Background

Hereditary angioedema (HAE) is a complement‑related disorder characterized by recurrent nonpruritic angioedema that can affect the skin, gastrointestinal tract, or airway. Acute HAE attacks—particularly laryngeal or pharyngeal swelling—can be life‑threatening and typically require prompt treatment with C1 esterase inhibitor replacement such as Berinert. Diagnosis and management are guided by specialist involvement (allergy/immunology, hematology, pulmonology, or medical genetics), avoidance of known triggers (including estrogen‑containing contraception or hormone therapy, ACE inhibitors or ARBs, DPP‑IV inhibitors, and neprilysin inhibitors), and laboratory confirmation of HAE subtype when applicable. Treatment decisions should follow the policy’s criteria and specialist consultation.

HAE Subtype Definitions

HAE I (C1-INH deficiency) criteria

C1-INH antigenic levelLow C1‑INH antigenic level (below the laboratory lower limit of normal).

C4 levelLow C4 level (below the laboratory lower limit of normal).

C1-INH functional levelLow C1‑INH functional level (below the laboratory lower limit of normal).

Supporting featuresFamily history of HAE or exclusion of acquired angioedema (e.g., symptom onset before age 30, normal C1q, no underlying disorders like lymphoma or MGUS).

HAE II (C1-INH dysfunction) criteria

C1-INH antigenic levelNormal to elevated C1‑INH antigenic level.

Revision History

2016-04-21effective_date

Policy initial effective date established for Berinert prior approval criteria.

2025-08-21last_reviewLatest

Policy last reviewed and revised (Last Revised Date reported as 08/21/2025).