CurrentColorado Rocky Mountain Health PlansPolicy 1 = 2025 P 2148-8

Haegarda (C1 esterase inhibitor, human) prior authorization/medical necessity

Defines clinical criteria and prior authorization requirements for coverage of Haegarda for routine prophylaxis of hereditary angioedema (HAE) for Colorado Rocky Mountain Health Plans members.

Policy Summary

PayerColorado Rocky Mountain Health Plans

PolicyHaegarda (C1 esterase inhibitor, human) prior authorization/medical necessity

Policy CodePolicy 1 = 2025 P 2148-8

Change TypeClarified — diagnostic and reauthorization language

Effective DateJun 1, 2025

Next Review Date

Key ActionDocument diagnosis with laboratory evidence or genetic/clinical confirmation and ensure prescription is for prophylaxis by an immunologist or allergist.

SourceLink

POLICY UPDATE CHANGES

Annual review with update to diagnostic criteria for HAE with normal C1 inhibitor levels and updated language for reauthorization criteria noted in 3/2024.

12 monthsAuthorization period

>=6 yrsMin age

7/2018 - 3/2025P&T history

1Program #

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Initial Authorization

Covered when ALL of the following are met:

ALL of the following

- C1 inhibitor (C1-INH) deficiency or dysfunction (Type I or II HAE) documented by laboratory evidence: C1-INH antigenic level below the lower limit of normal or C1-INH functional level below the lower limit of normal.
- HAE with normal C1 inhibitor levels and one of the following: confirmed pathogenic variant(s) in relevant genes (e.g., factor XII, angiopoietin‑1, plasminogen, kininogen‑1, myoferlin, HS3ST6); OR recurring angioedema attacks refractory to high‑dose antihistamines with a confirmed family history; OR recurring angioedema attacks refractory to high‑dose antihistamines with no known family history (de novo/HAE‑unknown).
Prescribed for prophylaxis of HAE attacks.
Not used in combination with other products indicated for prophylaxis against HAE attacks (e.g., Cinryze, Orladeyo, Takhzyro).
Prescriber is an immunologist or allergist.

Reauthorization

Covered when ALL of the following are met:

ALL of the following

Documentation of positive clinical response to Haegarda therapy.
Reduction in utilization of on‑demand therapies used for acute HAE attacks (e.g., Berinert, Firazyr, Ruconest) as determined by claims information while on Haegarda therapy.
Prescribed for prophylaxis of HAE attacks.
Not used in combination with other products indicated for prophylaxis against HAE attacks (e.g., Cinryze, Orladeyo, Takhzyro).
Prescriber is an immunologist or allergist.

Concomitant use with other prophylactic HAE products is not allowed.

Provider Actions & Authorization Guidance

Prior Authorization

Prior Authorization Required

Initial prior authorization is required. Provide diagnostic documentation confirming HAE and meet all prerequisites for prophylactic use of Haegarda. Authorization will be issued for 12 months when criteria are met.

Prescribed for prophylaxis of HAE attacks
Not used in combination with other prophylactic HAE agents (e.g., Cinryze, Orladeyo, Takhzyro)
Prescribed by an immunologist or allergist

Documentation Required

Initial Authorization Criteria — Diagnostic Confirmation & Prerequisites

Initial approval requires diagnostic confirmation of HAE by one of the following documented laboratory or genetic/clinical findings and fulfillment of prescriber and indication prerequisites.

Type I or II HAE: laboratory evidence of C1-INH deficiency/dysfunction — either C1‑INH antigenic level below the laboratory lower limit of normal OR C1‑INH functional level below the laboratory lower limit of normal
HAE with normal C1‑INH: one of the following — pathogenic variant(s) in genes associated with HAE (e.g., factor XII, angiopoietin‑1, plasminogen‑1, kininogen‑1, myoferlin, HS3ST6), OR recurring angioedema attacks refractory to high‑dose antihistamines with confirmed family history, OR recurring refractory attacks with unknown de‑novo mutation (HAE‑unknown)
Prescriber attestation that the patient has experienced attacks of severity/frequency warranting prophylactic therapy
Prescribed for prophylaxis and by an immunologist or allergist

Denial Risk

Combination Prophylactic Therapy Exclusion

Haegarda must not be used in combination with other agents indicated for HAE prophylaxis. Prior authorization will be denied if documentation shows concurrent prophylactic therapy with another HAE prophylactic product.

Combination prophylactic therapy with Cinryze, Orladeyo (berotralstat), Takhzyro (lanadelumab), or other prophylactic HAE agents is explicitly prohibited

Documentation Required

Reauthorization Criteria — Clinical Response & Utilization Metrics

Reauthorization requires documentation of continued benefit and reduced reliance on acute on‑demand treatments. Authorization will be issued for 12 months when reauthorization criteria are met.

Documentation of positive clinical response to Haegarda
Reduction in utilization of on‑demand therapies for acute attacks (e.g., Berinert, Firazyr, Ruconest) as evidenced by claims while on Haegarda
Prescribed for prophylaxis and not used in combination with other prophylactic HAE agents
Prescribed by an immunologist or allergist

Note

Automated Approval Note

Automated approval or re‑approval processes may be used in certain clinical programs and therapeutic classes. Coverage may be granted based solely on prior claims/medication history, diagnosis codes (ICD‑10), and claim logic where program rules permit.

UnitedHealthcare (or delegated program) may approve initial or reauthorization based on previous claim/medication history, ICD‑10 diagnosis codes, and/or claim logic
Use of automated approval processes varies by program/therapeutic class; supply limits may apply

Definitions

Type I/II HAE

DefinitionHereditary angioedema (HAE) Type I or II: C1 inhibitor (C1-INH) deficiency or dysfunction documented by laboratory evidence of C1-INH antigenic level below the lower limit of normal or C1-INH functional level below the lower limit of normal.

Laboratory evidence requiredC1-INH antigenic level below the lower limit of normal or C1-INH functional level below the lower limit of normal, per laboratory standards.

Associated terminologyReferred to in policy as Type I or II HAE when C1-INH antigenic or functional levels are low.

HAE with normal C1‑INH

DefinitionHAE with normal C1-INH: hereditary angioedema occurring despite normal C1 inhibitor levels and supported by either confirmed pathogenic variant(s) in specified genes or recurrent angioedema meeting clinical criteria.

Genetic confirmationConfirmed presence of variant(s) in genes such as factor XII, angiopoietin‑1, plasminogen‑1, kininogen‑1, myoferlin, or heparan sulfate‑glucosamine 3‑O‑sulfotransferase 6.

Clinical criteriaRecurring angioedema attacks refractory to high‑dose antihistamines with a confirmed family history of angioedema or with unknown de‑novo mutation(s) (HAE‑unknown).

Revision History

2018-07creation

Program created (Change Control, new prior authorization program for Haegarda).

2019-07annual_review

New program entry recorded (program documentation updated).

2020-06annual_review

Annual review; criteria aligned with acute and prophylactic therapies.

2021-03update

Added diagnostic criteria and aligned combination use language with prophylactic therapies; updated references.

2022-03annual_review

Annual review with updates to background and references.

2023-03annual_review

Annual review; updated combination use language with prophylactic therapies without change to clinical intent.

2024-03annual_review

Annual review with update to diagnostic criteria for HAE with normal C1 inhibitor levels and updated language for reauthorization criteria.

2025-03annual_reviewLatest

Annual review; no changes to the clinical criteria.

OpenPayer

Haegarda (C1 esterase inhibitor, human) prior authorization/medical necessity

Coverage Criteria

Provider Actions & Authorization Guidance

Background

Definitions

Revision History