CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Berinert (C1 esterase inhibitor [human]) (Intravenous)

Clinical medical policy governing prior authorization and coverage criteria for Berinert (C1 esterase inhibitor [human]) administered intravenously for treatment of hereditary angioedema (HAE) for Medicaid, Commercial, and Medicare members (medication on the medical benefit).

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyBerinert (C1 esterase inhibitor [human]) (Intravenous)

Policy CodePolicy N/A

Change TypeNo material change

Effective Date01/01/2020

Next Review Date

Key ActionPrior authorization is required and requests must meet the policy's initial criteria including age, prescriber specialty/consult, diagnostic confirmation, and other documentation.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 yearsmin age

20 IU/kgacute dose

1100 unitsmax per 28d

J0597HCPCS

6 monthscoverage duration

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Coverage Criteria for Berinert (C1 Esterase Inhibitor [Human])

Initial Therapy

Covered when ALL of the following are met:

Initial Criteria: Member is 6 years of age or older; AND medication is prescribed by, or in consultation with, an allergist/immunologist or a physician who specializes in the management of HAE; AND Berinert is being used for treatment of acute hereditary angioedema (HAE) attacks; AND member has history of moderate to severe cutaneous attacks (without concomitant hives) OR abdominal attacks OR mild to severe airway swelling attacks of HAE (i.e., debilitating cutaneous/gastrointestinal symptoms OR laryngeal/pharyngeal/tongue swelling);

Diagnostic confirmation: Member has documented diagnosis of HAE type I or type II and meets one of the following: (1) C1 inhibitor (C1-INH) antigenic level below the laboratory lower limit of normal, OR (2) normal C1-INH antigenic level with low C1-INH functional level (functional C1-INH <50% or below lab lower limit); OR member has normal C1-INH confirmed by laboratory testing AND either a pathogenic genetic variant (e.g., F12, ANGPT1, PLG, KNG1, HS3ST6, MYOF) OR documented family history of angioedema with the member's angioedema refractory to a trial of high‑dose antihistamine therapy (e.g., cetirizine 40 mg/day or equivalent) for at least one month;

Concomitant therapy and exclusions: Requested medication will not be used in combination with other products indicated for acute treatment of HAE attacks (e.g., Ekterly, Kalbitor, Ruconest, or Icatibant); AND other causes of angioedema have been ruled out (e.g., ACE‑inhibitor induced angioedema, estrogen‑related angioedema, allergic angioedema);

Step therapy for Commercial/Medicaid: For Commercial and Medicaid members, failure, contraindication, or intolerance to Ruconest is required prior to approval; Medicare members who have received this medication within the past 365 days are not subject to step therapy requirements.

Continuation Therapy

Continuation of therapy is covered when ALL of the following are met:

Continuation Criteria: Member continues to meet initial criteria; AND documentation shows reduction in severity and duration of attacks since starting treatment; AND consideration of prophylactic therapy is based on attack frequency, attack severity, comorbid conditions, and the member's quality of life.

Investigational use: Therapies are considered investigational when used at a dose or for a condition other than those recognized as medically accepted indications in standard reference compendia (for example, AHFS‑DI, Micromedex DrugDex, Clinical Pharmacology, Lexi‑Drugs, or peer‑reviewed literature). Neighborhood does not provide coverage for drugs used for investigational purposes.

Berinert is covered under the Medical Benefit only when used within the policy guidelines. Use outside of these guidelines may result in non‑payment unless an exception is approved. Coverage decisions will be reviewed prospectively via the prior authorization process.

Provider Actions, Prior Authorization, and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required and requests are reviewed prospectively through the prior authorization process. Coverage will be determined based on the criteria in this policy; use outside these guidelines may result in non-payment unless approved through an exception process.

Prior authorization required — requests reviewed prospectively
Non-covered without approved exception

Step Therapy

Step Therapy Requirement

For Commercial and Medicaid members, step therapy requires a trial and documented failure, contraindication, or intolerance to Ruconest prior to approval. Medicare members who have received this medication within the past 365 days are not subject to the step therapy requirement.

Commercial & Medicaid: trial/failure/contraindication/intolerance to Ruconest required
Medicare: exemption if previously received medication within past 365 days

Documentation Required

Documentation Required for Prior Authorization

Prior authorization requests must include documentation to support medical necessity: age of the member, specialty of the prescribing or consulting clinician (allergist/immunologist or HAE specialist), documented diagnosis of HAE type I or II with supporting laboratory or genetic testing, history and description of attack type/severity (cutaneous, abdominal, airway), documentation that other causes of angioedema have been ruled out, confirmation that Berinert will not be used in combination with other acute HAE products, and for Commercial/Medicaid members evidence of trial/failure of Ruconest. For continuation requests include documentation of reduction in severity and duration of attacks since starting treatment.

Required documents: member age
Required documents: specialty/consultation (allergist/immunologist or HAE specialist)
Required documents: documented HAE diagnosis (type I or II) with lab or genetic test results
Required documents: attack history and severity (cutaneous, abdominal, airway)
Required documents: documentation other causes of angioedema ruled out
Required documents: statement medication not used with other acute HAE products
Commercial/Medicaid: documentation of Ruconest trial/failure/contraindication/intolerance
Continuation: documentation of reduced attack severity/duration

Denial Risk

Non-compliant Use May Be Denied

Use outside of the policy criteria (non-compliant use) may be denied. Providers may request an exception or submit supporting documentation for review if the requested use falls outside these guidelines.

Non-compliant use may be denied without an approved exception
Providers may submit exception requests with supporting documentation for review

Definitions

HAE type I or II

DefinitionHereditary angioedema (HAE) type I or II: HAE with C1 inhibitor (C1-INH) deficiency or dysfunction confirmed by laboratory testing

Diagnostic criteria (antigenic)C1-INH antigenic level below the laboratory lower limit of normal

Diagnostic criteria (functional)Normal antigenic level with low functional C1-INH (<50% or below lab lower limit of normal)

HAE with normal C1-INH

DefinitionHAE with normal C1-INH: HAE with normal C1 inhibitor levels confirmed by laboratory testing and associated with documented pathogenic genetic variants or compatible family history

Genetic variants examplePathogenic variants may include F12, ANGPT1, PLG, KNG1, HS3ST6, or MYOF as confirmed by genetic testing

Alternative diagnostic pathwayDocumented family history of angioedema and angioedema refractory to a trial of high-dose antihistamine therapy (e.g., cetirizine 40 mg/day for ≥1 month)

OpenPayer

Berinert (C1 esterase inhibitor [human]) (Intravenous)

Coverage Criteria for Berinert (C1 Esterase Inhibitor [Human])

Coding and Dosing

Provider Actions, Prior Authorization, and Documentation

Background

Definitions