Berinert (C1 Esterase Inhibitor) prior authorization for Hereditary Angioedema
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Defines prior authorization and step-edit requirements for Berinert (J0597) for treatment of acute attacks and prophylaxis of hereditary angioedema (HAE) for AvMed members; applies to prescribers and administrators requesting coverage.
No material clinical or coverage changes in this revision.
Coverage Criteria for Berinert (C1 Esterase Inhibitor)
Initial Authorization Criteria
Covered when ALL of the following are met:
From checklist items and initial authorization duration
From member history/clinical presentation bullets
Detailed subtype-specific laboratory and history requirements; see checklist for required supporting documentation
Step-therapy / prior trials requirement and icatibant trial when specified
Reauthorization
Reauthorization requirements
Reauthorization period: 12 months; documentation including lab results, diagnostics, and chart notes must be provided
The use of medication samples to start therapy does not satisfy AvMed's step-edit or prior authorization requirements. Providers should not rely on sample availability as meeting authorization criteria; formal prior authorization with required documentation must be submitted for coverage determination.
Requests that do not include required laboratory confirmation of the HAE subtype, documentation of specified prior trials (when applicable), or complete supporting records may be delayed or denied. The policy requires subtype-consistent testing (with repeat blood testing as applicable), evidence of trial and failure of indicated prophylactic therapies for certain presentations, and submission of chart notes and lab results to confirm checklist items.
To avoid denials or delays, include repeat blood testing during an attack when pursuing a diagnosis of HAE III, documentation of trial and failure of icatibant when required, and evidence of inadequate response or intolerance to prophylactic agents (e.g., tranexamic acid, aminocaproic acid, danazol, or progestins) where specified.
Coding and Dosing Information
| J0597 | Berinert, C1 esterase inhibitor (human) (500 unit vial) - HCPCS |
| NDC 63833-0825-xx | Example NDC for 500 unit vial |
Provider Actions and Submission Requirements
Prior authorization required — submit AvMed PA/Step-Edit form
Prior authorization is required for Berinert (J0597) medical benefit; submit the AvMed Medical Prior Authorization/Step-Edit Request form with required signatures and documentation.
- Drug Requested: Berinert (J0597) (Medical) must be requested via the form
- Submit with all required supporting documentation to avoid delay
Step therapy — required prior trials for HAE III
For HAE III with normal C1-INH, the member must have had an inadequate response or intolerance to an adequate trial of prophylactic therapies (e.g., tranexamic acid, aminocaproic acid, danazol, or progestins for females) before approval; trial and failure of icatibant is required when specified elsewhere and documentation must be submitted.
- Document an inadequate response or intolerance to one listed prophylactic agent for HAE III
- Icatibant trial and failure is required when indicated (submit prior authorization documentation)
Required supporting documentation — labs, diagnostics, chart notes, genetic/family history
Submit documentation including lab results, diagnostics, and chart notes to support each checklist item; provide HAE subtype confirmation with repeat blood testing as applicable, prior therapy trials/failures (including icatibant when required), and family history or genetic testing evidence when applicable.
- Laboratory values confirming HAE subtype (C1-INH antigenic level, C4, C1-INH functional level) and repeat testing during an attack for HAE III
- Chart notes documenting symptoms, frequency/severity of attacks, and prior therapy trials and outcomes
- Genetic testing or family history documentation for known HAE-causing mutations when applicable
Form completion requirements — signature and required identifiers/data
The prescriber must sign and clearly print their name on the AvMed PA/Step-Edit Request form; include member identifiers, prescriber identifiers (including NPI), diagnosis and ICD code, dosing, weight and date weight obtained, and contact information to avoid authorization delays.
- Prescriber signature and printed name (preprinted stamps not valid)
- Member name and AvMed number, prescriber NPI, office contact, phone and fax
- Diagnosis and ICD code, drug form/strength, dosing schedule, length of therapy, weight and date weight obtained
Incomplete or illegible submissions may delay or deny authorization
Authorization can be delayed or denied if the PA form information is incomplete, incorrect, or illegible; incomplete checklist items or missing supporting documentation may lead to denial.
- Ensure all checklist lines are supported by lab results, diagnostics, and chart notes as requested on the form
- Use of samples to initiate therapy does not meet step-edit/preauthorization criteria and may affect decision
Background
Hereditary angioedema (HAE) is a disorder characterized by recurrent angioedema attacks. Many cases result from C1 esterase inhibitor (C1‑INH) deficiency or dysfunction (HAE types I and II), while type III presents with normal C1‑INH laboratory values and other genetic causes. Clinical presentations prompting consideration of therapy include frequent or severe attacks (for example, three (3) or more severe attacks per month), cutaneous attacks without hives, abdominal attacks, airway swelling, or disabling symptoms that impair functioning.
Diagnosis and subtype confirmation depend on laboratory testing: HAE I typically shows low C1‑INH antigenic level, low C4, and low C1‑INH functional level; HAE II shows normal/elevated C1‑INH antigenic level with low C4 and low functional level; HAE III is characterized by normal C1‑INH antigenic level, normal C4, and normal C1‑INH functional level and requires repeat testing during an attack and additional evidence such as a known HAE‑causing mutation or documented inadequate response to prophylactic therapies. Berinert (a plasma‑derived C1 esterase inhibitor) is used for acute attack treatment and for prophylaxis in specified circumstances.
Definitions and Subtypes
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