CurrentViva Health PlanPolicy IC-0018

Berinert (C1 Esterase Inhibitor, Human) Intravenous Therapy for Acute Hereditary Angioedema Attacks

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This policy governs prior authorization, medical necessity, dosing, renewal, and billing requirements for intravenous Berinert used to treat acute hereditary angioedema attacks.

Policy Summary

PayerViva Health Plan

PolicyBerinert intravenous therapy for acute hereditary angioedema attacks

Policy CodePolicy IC-0018

Change TypeRoutine reviewno material change

Effective DateN/A

Last ReviewOct 1, 2026

Next Review DateN/A

Key ActionObtain prior authorization and submit documentation confirming specialist involvement and a qualifying hereditary angioedema diagnosis before treatment.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 weeksInitial authorization

12 weeksRenewal interval

1,000 unitsMaximum per 28 days

20 IU/kgDose per acute attack

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CurrentViva Health PlanPolicy IC-0018

Berinert (C1 Esterase Inhibitor, Human) Intravenous Therapy for Acute Hereditary Angioedema Attacks

Customize your policy alerts

Sign up for vivahealthplan Policy IC-0018 alerts

Get alerted when Policy IC-0018 changes without checking for updates manually.

Monitor payer policy activity

Receive email alerts as soon as relevant payer policies are published or updated.

This policy governs prior authorization, medical necessity, dosing, renewal, and billing requirements for intravenous Berinert used to treat acute hereditary angioedema attacks.

Policy Summary

PayerViva Health Plan

PolicyBerinert intravenous therapy for acute hereditary angioedema attacks

Policy CodePolicy IC-0018

Change TypeRoutine reviewno material change

Effective DateN/A

Last ReviewOct 1, 2026

Next Review DateN/A

Key ActionObtain prior authorization and submit documentation confirming specialist involvement and a qualifying hereditary angioedema diagnosis before treatment.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 weeksInitial authorization

12 weeksRenewal interval

1,000 unitsMaximum per 28 days

20 IU/kgDose per acute attack

D84.1Covered diagnosis

Coverage Criteria

Initial therapy for acute HAE attacks

Covered when all universal criteria and at least one qualifying acute HAE diagnostic pathway are met.

Universal criteria and acute HAE indication

Universal criteria
- Specialist prescribing: Berinert is prescribed by, or provided in consultation with, a specialist in allergy, immunology, hematology, pulmonology, or medical genetics.
- No duplicate acute HAE therapy: Berinert will not be used in combination with another agent indicated for treatment of acute HAE attacks. Requests for duplicate combination therapy may be reviewed case by case.
- Trigger counseling: The provider confirms that the member has been counseled to avoid possible medication triggers for HAE attacks, including estrogen-containing medications and ACE inhibitors.
ONE qualifying acute HAE diagnostic pathway
- HAE-C1-INH Type 1 or 2: The member has HAE due to C1 inhibitor deficiency, Type 1 or Type 2, and at least one of the following is present: current or historical C1-INH antigenic level below the performing laboratory's lower limit of normal; current or historical C1-INH functional level below the performing laboratory's lower limit of normal; or pathogenic mutation(s) in SERPING1.
- HAE with normal C1-INH and known mutation: The member has HAE with normal C1-INH, a current or historical normal C1-INH level as defined by the performing laboratory, and a confirmed HAE-causing mutation in F12, ANGPT1, PLG, KNG1, MYOF, and/or HS3ST6.
- HAE with normal C1-INH and antihistamine-refractory attacks: The member has recurrent angioedema attacks without hives that are refractory to a 4-week trial of high-dose antihistamines.4 weeks at 4 times the standard dose
  High-dose antihistamine therapy means a second-generation H1 antagonist at four times the standard dose.

Continuation therapy

Renewal may be authorized every 12 weeks when all continuation requirements are met.

Continuation requirements

Ongoing eligibility: The member continues to meet the applicable universal and indication-specific criteria for acute HAE treatment.
Clinical benefit: The member has experienced clinical benefit from therapy, such as reduced attack severity or shortened attack duration.
Tolerability: The member has no unacceptable toxicity from Berinert, including severe hypersensitivity reactions or serious arterial or venous thromboembolic events.
Cumulative medication supply: The member's cumulative on-hand supply of medication indicated for the acute treatment of HAE is considered when authorization is determined.

Concurrent acute HAE therapy: Berinert should not be used in combination with another agent indicated for treatment of acute hereditary angioedema attacks. Requests for duplicate combination therapy may be considered and are reviewed on a case-by-case basis.

Other causes of angioedema: Coverage is not intended for angioedema caused by another condition, including drug-induced angioedema, mast cell-mediated angioedema, or acquired angioedema due to C1-INH deficiency. These alternative causes must be ruled out before hereditary angioedema is confirmed for coverage.

Coding and Billing

Covered HCPCS CodesHCPCSCovered

J0597

Injection, c-1 esterase inhibitor (human), berinert, 10 units

Covered Diagnosis CodesICD-10Covered

D84.1

Defects in the complement system

Covered NDC CodesNDCCovered

63833-0825-xx	Berinert 500 IU single-dose carton kit containing a single-dose vial of Berinert and a 10 mL vial of Sterile Water for Injection
63833-0835-xx	Berinert 500 IU single-dose vial

Covered Diagnosis CodesICD-10Covered

D84.1

Defects in the complement system

Dosing Limit

Maximum dosing limit1,000 billable units per 28 days

C1-INH Laboratory Confirmation

HAE-C1-INH laboratory thresholdC1-INH antigenic or functional level below the lower limit of normal as defined by the performing laboratory

Antihistamine Trial

High-dose antihistamine trialFour weeks at four times the standard dose of a second-generation H1 antagonist

Provider Actions and Authorization

Prior Authorization

Initial Prior Authorization Required

Prior authorization is required. Initial authorization for Berinert is valid for 12 weeks. The approved quantity must account for the member’s cumulative supply of acute HAE medication and support treatment of up to 4 acute attacks per 4 weeks during the authorization period.

Prior Authorization

Renewal Authorization Every 12 Weeks

Renewal prior authorization may be issued every 12 weeks after the initial authorization period. Renewal requests must satisfy the applicable renewal criteria and account for the member’s cumulative on-hand acute HAE medication.

Unsupported block type: info

Unsupported block type: documentation

Documentation Required

Clinical Background

Hereditary angioedema (HAE) is a recurrent disorder that can produce episodes of swelling involving the abdomen and upper airway, including the mouth, tongue, and larynx. Acute attacks may present with severe abdominal pain, nausea, vomiting, diarrhea, or prodromal symptoms such as anxiety, paresthesia, and fatigue; attacks may also occur in members with a positive family history of HAE.

Definitions

HAE-C1-INH

DefinitionHereditary angioedema due to C1 inhibitor deficiency, Type 1 or Type 2

Laboratory confirmationCurrent or historical C1-INH antigenic level below the laboratory lower limit of normal, or C1-INH functional level below the laboratory lower limit of normal

Genetic confirmationPathogenic mutation(s) in SERPING1 confirmed by genetic testing

HAE-nC1-INH

DefinitionHereditary angioedema with normal C1-INH

Laboratory requirementCurrent or historical normal C1-INH level as defined by the performing laboratory

Genetic support

Initial Therapy Criteria

Initial treatment for acute HAE

Initial therapy is authorized when all universal criteria and one qualifying acute HAE diagnostic pathway are met.

Initial treatment requirements

Universal criteria
- Specialist prescribing: Berinert is prescribed by, or provided in consultation with, a specialist in allergy, immunology, hematology, pulmonology, or medical genetics.
- No duplicate acute HAE therapy: Berinert will not be used in combination with another agent indicated for treatment of acute HAE attacks. Requests for duplicate combination therapy may be reviewed case by case.
- Trigger counseling: The provider confirms that the member has been counseled to avoid possible medication triggers for HAE attacks, including estrogen-containing medications and ACE inhibitors.

Continuation Criteria

Renewal criteria

Renewal may be authorized every 12 weeks when all continuation requirements are met.

Berinert renewal requirements

Ongoing eligibility: The member continues to meet the applicable universal and indication-specific criteria for acute HAE treatment.
Clinical benefit: The member has experienced clinical benefit from therapy, such as reduced attack severity or shortened attack duration.
No unacceptable toxicity: The member has no unacceptable toxicity from Berinert, including severe hypersensitivity reactions or serious arterial or venous thromboembolic events.

Step Therapy

Step	Requirement	Outcome
1	For the HAE with normal C1-INH clinical pathway, the member must have recurrent angioedema attacks without hives that remain refractory to a 4-week trial of a second-generation H1 antagonist at four times the standard dose.	Required before coverage under this pathway

Quantity Limits

Berinert Quantity Limit

Maximum quantity1,000 billable units

Time period28 days

Acute HAE Medication Supply

Cumulative supplySufficient quantity of acute HAE medication, including Berinert, to treat up to 4 acute attacks

Review periodPer 4 weeks for the duration of the authorization

Site of Care and Administration

Note

Provide instruction for home administration

Administer Berinert by intravenous injection. Members may self-administer upon recognition of HAE attack symptoms after receiving instruction from their healthcare provider.

Recommended dose: 20 international units (IU) per kg body weight.
Self-administration is permitted after provider instruction.

Policy Summary

PayerViva Health Plan

PolicyBerinert intravenous therapy for acute hereditary angioedema attacks

Policy CodePolicy IC-0018

Change TypeRoutine reviewno material change

Effective DateN/A

Last ReviewOct 1, 2026

Next Review DateN/A

Key ActionObtain prior authorization and submit documentation confirming specialist involvement and a qualifying hereditary angioedema diagnosis before treatment.

SourceLink

On This Page

Sufficient supply to treat up to 4 acute attacks per 4 weeks

HAE Diagnosis and Supporting Records

Submit documentation supporting a qualifying HAE history, exclusion of other causes of angioedema, and confirmed HAE diagnosis. The records must establish the applicable clinical and laboratory, genetic, or treatment-response criteria.

Qualifying history may include recurrent swelling of mucous membranes, skin, or subcutaneous tissue; recurrent abdominal attacks with severe abdominal pain, nausea, vomiting, or diarrhea; upper-airway edema; prodromal symptoms; or a positive family history.
Rule out other causes, including drug-induced angioedema, mast cell-mediated angioedema, and acquired angioedema due to C1-INH deficiency.
For HAE-C1-INH type 1 or 2: document a current or historical C1-INH antigenic level below the laboratory lower limit of normal, a C1-INH functional level below the lower limit of normal, or pathogenic SERPING1 mutation(s).
For HAE with normal C1-INH: document a normal C1-INH level plus a known HAE-causing mutation in F12, ANGPT1, PLG, KNG1, MYOF, or HS3ST6; alternatively, document recurrent angioedema without hives refractory to a 4-week trial of high-dose second-generation H1 antihistamine therapy (four times the standard dose).

Unsupported block type: documentation

Unsupported block type: billing_rule

Denial Risk

Duplicate Acute HAE Therapy

Berinert must not be used concurrently with another agent indicated for treatment of acute HAE attacks. Requests for combination duplicate therapy may be considered on a case-by-case basis, but concurrent duplicate therapy creates denial risk when not specifically justified.

Document the clinical rationale when requesting combination acute HAE therapy.
Do not submit duplicate acute HAE therapy without case-specific justification.

Unsupported block type: denial_risk

Confirmed HAE-causing mutation(s) in F12, ANGPT1, PLG, KNG1, MYOF, and/or HS3ST6

Clinical supportRecurrent angioedema attacks without hives refractory to a four-week trial of high-dose antihistamines at four times the standard dose

Non-Quantitative Treatment Limitation (NQTL)

DefinitionMethods, guidelines, standards of evidence, or other conditions that can restrict how long or to what extent benefits are provided under a health plan

ExamplesUtilization review or prior authorization

Prior authorization factorsTreatment indication and cost of the drug are considered for prior authorization

ONE qualifying acute HAE diagnostic pathway

HAE-C1-INH Type 1 or 2: The member has HAE due to C1 inhibitor deficiency, Type 1 or Type 2, and at least one of the following is present: current or historical C1-INH antigenic level below the performing laboratory's lower limit of normal; current or historical C1-INH functional level below the performing laboratory's lower limit of normal; or pathogenic mutation(s) in SERPING1.
HAE with normal C1-INH and known mutation: The member has HAE with normal C1-INH, a current or historical normal C1-INH level as defined by the performing laboratory, and a confirmed HAE-causing mutation in F12, ANGPT1, PLG, KNG1, MYOF, and/or HS3ST6.
HAE with normal C1-INH and antihistamine-refractory attacks: The member has recurrent angioedema attacks without hives that are refractory to a 4-week trial of high-dose antihistamines.4 weeks at 4 times the standard dose
High-dose antihistamine therapy means a second-generation H1 antagonist at four times the standard dose.

Cumulative on-hand supply:

The member's cumulative on-hand supply of medication indicated for the acute treatment of HAE is considered when authorization is determined.

Sufficient supply to treat up to 4 acute attacks per 4 weeks