CurrentCignaPolicy v010126

Berinert and Cinryze prior authorization for hereditary angioedema

Cigna prior-authorization form and criteria for coverage of Berinert 500 unit kit and Cinryze 500 unit vial for treatment or prophylaxis of hereditary angioedema (HAE); intended for prescribing providers, specialty pharmacies, and dispensing facilities submitting requests to Cigna.

Policy Summary

PayerCigna

PolicyBerinert and Cinryze prior authorization for hereditary angioedema

Policy CodePolicy v010126

Change TypeMinor / no material changes

Effective Date

Next Review Date

Key ActionSubmit prior authorization with required documentation including diagnostic labs (functional C1‑INH and serum C4), indication, weight (for Berinert), ICD-10 and supporting medical records.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

2drugs listed on form

5 business daysstandard review time

1urgent phone number

HAE type I, II, IIIdiagnosis options

Coverage Criteria for Berinert and Cinryze

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Coverage criteria by indication and HAE subtype

Coverage assessment is performed according to HAE subtype (C1‑INH deficiency type I/II vs HAE with normal C1‑INH/type III), indication (prophylaxis vs treatment), and prior approval status; required documentation differs by scenario.

Initial therapy for HAE due to C1-INH deficiency (type I or II): Patient has HAE due to C1‑INH deficiency (type I or II) AND documentation is provided confirming low functional C1‑INH (less than 50% of normal) at baseline and low serum C4 at baseline, OR the patient is currently receiving therapy without prior Cigna approval (treat as initial therapy). Attach supporting medical documentation (chart notes, laboratory results, genetic test results if applicable).functional C1-INH <50%

Medical documentation specific to your response must be attached or request may be denied.

Continuation therapy for HAE due to C1-INH deficiency (type I or II): Patient is currently receiving Berinert or Cinryze for the requested indication with prior Cigna approval AND documentation is provided confirming the HAE (type I or II) diagnosis AND prescriber documents a favorable clinical response since initiating therapy (examples: decreased attack frequency, severity, or duration).documented favorable clinical response

If the patient is currently receiving the requested therapy but has not previously received approval for this specific indication through Cigna, answer 'No' to prior approval and review as Initial Therapy.

HAE with normal C1-INH (type III) — treatment or prophylaxis: For HAE with normal C1‑INH (type III), documentation must confirm normal C1‑INH levels (protein and/or function) and normal serum C4 per laboratory reference values. When available, genetic testing documenting a pathogenic variant in one of the listed genes (F12, PLG, ANGPT1, KNG1, MYOF, HS3OST6) is requested. Prescriber should be an allergist/immunologist or HAE specialist (or in consultation) and document response to high‑dose oral H1 antihistamine therapy when applicable. NOTE: A diagnosis of HAE with normal C1‑INH does NOT satisfy the requirements for HAE due to C1‑INH deficiency (type I/II).genetic confirmation when available

Attach patient‑specific documentation; requests without required documentation may be denied.

Berinert for acute treatment-specific considerations: If requesting Berinert for treatment of an acute HAE attack (HAE due to C1‑INH deficiency), prescriber should document a history of at least one laryngeal attack OR document allergy to rabbits/rabbit-derived products. If neither is present, documentation that the patient has tried Ruconest may be requested; Ruconest is identified as the preferred product in that scenario.history of laryngeal attack OR Ruconest trial documented

If Ruconest has not been tried and no contraindication exists, reviewer may consider Ruconest as preferred product.

What Providers Must Do

Prior Authorization

Prior Authorization Required

Prior authorization is required for Berinert and Cinryze. Complete the prior authorization form fields (medication, indication — treatment or prophylaxis, directions, quantity, duration, J-code, patient weight where applicable) and submit via the online portal (CoverMyMeds or SureScripts) or by fax as directed.

Medications affected: Berinert (500 unit kit), Cinryze (500 unit vial)
Indication must be specified: treatment vs prophylaxis
If patient is currently receiving therapy but lacks prior approval for this specific indication, treat as Initial Therapy

Denial Risk

Requests May Be Denied Without Required Documentation

Requests may be denied if required medical documentation is not provided. Provide patient-specific documentation with identifying information; lack of necessary records (chart notes, lab reports, prior approvals) can result in denial.

Denial risk if documentation confirming HAE diagnosis or prior Cigna approvals is missing
Ensure documentation includes patient identifiers and dates

Documentation Required

Attach Patient-Specific Documentation

Attach all relevant patient-specific documentation to the request. Acceptable materials include chart notes, laboratory results (C1‑INH protein/function, serum C4), genetic testing results, prior authorization approvals, claims records, prescription receipts, and other medical test results. Documentation must confirm the HAE subtype (type I or II for C1‑INH deficiency) when applicable and include patient identifiers.

For C1‑INH deficiency (type I or II): attach functional C1‑INH results showing <50% of normal and/or low serum C4 at baseline
For HAE with normal C1‑INH (type III): attach C1‑INH protein/function and C4 confirming normal values, and genetic test results if available
If previously approved by Cigna, attach prior approval documentation and evidence of clinical response

Step Therapy

Preferred Product / Step Therapy

Step-therapy / preferred product note: Ruconest is the preferred product for some treatment scenarios. If requesting Berinert for treatment and Ruconest has not been tried, provide documentation explaining why Ruconest is not appropriate or document that Ruconest was trialed. For prophylactic use or if a history of laryngeal attack exists, follow the criteria and include documentation of prior treatments and clinical response.

If Berinert is requested for treatment and Ruconest has not been tried, indicate whether Ruconest was attempted
If patient has allergy to rabbits/rabbit-derived products or other contraindication, document this
Document history of laryngeal attacks when relevant

Background

The form collects clinical details to confirm the type of hereditary angioedema (HAE) and to determine applicable criteria. It captures whether the patient has HAE due to C1‑INH deficiency (type I or II) versus HAE with normal C1‑INH (type III), whether the request is for treatment of an acute attack or for prophylaxis, and prior approval status. For C1‑INH deficiency, the form requests baseline laboratory evidence (functional C1‑INH and serum C4); for HAE with normal C1‑INH the form requests confirmation of normal C1‑INH/C4 and, when available, genetic testing or documentation of response to high‑dose H1 antihistamines.

Definitions

HAE with normal C1‑INH (type III) — definition and documentation requirements

DefinitionHereditary angioedema with normal C1‑INH (HAE type III) — a phenotype in which C1 esterase inhibitor protein level and/or functional activity are within laboratory reference (normal) range.

Required laboratory confirmationAttach documentation confirming normal C1‑INH (protein level and/or functional activity) as defined by the laboratory reference values.

Serum C4 requirementAttach documentation confirming normal serum C4 level, as defined by the laboratory reference values.

Genetic testingWhen available, provide documentation of a pathogenic variant in one of the listed genes (F12, PLG, ANGPT1, KNG1, MYOF, HS3OST6).

Indication options on formForm captures whether the request is for treatment of acute HAE type III attacks or for prophylaxis of HAE type III attacks.

Documentation noteMedical documentation (chart notes, lab results, genetic test results, etc.) with patient identifiers must be attached or the request may be denied.

OpenPayer

Berinert and Cinryze prior authorization for hereditary angioedema

Coverage Criteria for Berinert and Cinryze

Billing / Code Fields

What Providers Must Do

Background

Definitions