CurrentCignaPolicy N/A

Hereditary Angioedema - Takhzyro Prior Authorization Policy

Defines Cigna's prior authorization requirements, coverage criteria, continuation criteria, and exclusions for Takhzyro (lanadelumab-flyo) for prophylaxis of hereditary angioedema (HAE) due to C1-INH deficiency in patients ≥ 2 years of age.

Policy Summary

PayerCigna

PolicyHereditary Angioedema - Takhzyro Prior Authorization Policy

Policy CodePolicy N/A

Change TypeCriteria clarification and additions

Effective Date

Next Review Date

Key ActionObtain prior authorization and document baseline functional C1‑INH (<50% of normal) and serum C4 below reference range; ensure prescription is by or in consultation with an allergist/immunologist or HAE specialist.

POLICY UPDATE CHANGES

Added statement that a person who previously met initial therapy criteria under the Coverage Review Department and is currently receiving Takhzyro only needs to meet continuation criteria.

Deleted '[Type I or Type II]' from the indication heading and clarified wording around diagnosis ('type' before II) and prior approvals from other entities.

Added note under 'Patient is currently receiving Takhzyro prophylaxis' that patients must meet initial therapy criteria and approval through the Coverage Review Department if they had previously received initial therapy approval through another entity.

1Approval Duration (years)

2+Approved Age (years)

1Covered Indication (HAE due to C1-INH deficiency)

Coverage Summary

Scope: Defines Cigna's prior authorization requirements, coverage criteria, continuation criteria, and exclusions for Takhzyro (lanadelumab‑flyo) for prophylaxis of hereditary angioedema (HAE) due to C1‑INH deficiency in patients ≥ 2 years of age. (Cigna approvals are provided for 1 year.)

Overview note: Takhzyro is indicated for long‑term prophylaxis to prevent HAE attacks in patients aged 2 years and older. Prior authorization is required; prescription must be by or in consultation with an allergist/immunologist or HAE specialist. Continuation approvals require documentation of diagnosis and clinical benefit or prior approval under the Coverage Review Department.

Key Coverage Facts

IndicationProphylaxis of HAE due to C1-INH deficiency

Minimum Age≥ 2 years

Approval Duration1 year

Coverage Stancecovered_with_criteria

Medical Necessity / Coverage Criteria

FDA-Approved Indication - Hereditary Angioedema (HAE) Due to C1-INH Deficiency - Prophylaxis

Approve Takhzyro for 1 year if the patient meets ONE of the following (A or B):

A) Initial therapy

ALL of the following
- Patient has low levels of functional C1-INH protein (< 50% of normal) at baseline, as defined by the laboratory reference values< 50% of normal
  [documentation required]
- Patient has lower than normal serum C4 levels at baseline, as defined by the laboratory reference values
  [documentation required]
Medication is prescribed by or in consultation with an allergist/immunologist or a physician who specializes in the treatment of HAE or related disorders

Provider Actions & Prior Authorization

Prior Authorization

Prior authorization required

Prior authorization is recommended/required for Takhzyro; approvals are provided for 1 year and require prescription by or in consultation with an allergist/immunologist or HAE specialist.

Documentation Required

Diagnostic laboratory documentation

Provide documentation of baseline laboratory values: functional C1-INH level (< 50% of normal) and serum C4 level below laboratory reference ranges; chart notes, lab records, and prescription claims may be required.

Coding

DrugNDCCovered

No codes listed

Background and Evidence

Background: Takhzyro is a human monoclonal antibody inhibitor of plasma kallikrein indicated for prophylaxis to prevent hereditary angioedema (HAE) attacks in patients ≥ 2 years of age.

Guideline and diagnostic recommendations: US HAEA (2020) recommends measuring serum C4, C1‑INH antigenic level, and C1‑INH functional level when HAE is suspected; low C4 plus low C1‑INH antigenic or functional level is consistent with HAE types I/II. The WAO/EAACI (2021) guideline likewise supports evaluation for long‑term prophylaxis at each visit and endorses first‑line options including plasma‑derived C1‑INH and Takhzyro.

Coverage criteria summary / first‑line options and evidence: First‑line prophylactic therapies for HAE types I/II include intravenous or subcutaneous C1‑INH products (e.g., Cinryze, Haegarda) and Takhzyro. FDA prescribing information and guideline endorsements (US HAEA 2020; WAO/EAACI 2021) support Takhzyro as a first‑line option for long‑term prophylaxis. Documentation required for coverage includes baseline labs showing functional C1‑INH protein < 50% of normal and low serum C4, or for patients already on Takhzyro, a diagnosis of HAE type I/II plus evidence of a favorable clinical response since initiating therapy.

Definitions

TermHAE with normal C1-INH (HAE type III)

DefinitionA form of hereditary angioedema that does NOT satisfy the policy's diagnostic requirement for Takhzyro coverage for types I/II.

Revision History

2024-10-09addedLatest

Added statement that a person who previously met initial therapy criteria under the Coverage Review Department and is currently receiving Takhzyro only needs to meet continuation therapy criteria; if past criteria have not been met under the Coverage Review Department and the patient is currently receiving Takhzyro, initial therapy criteria must be met.

2024-10-09revised

Deleted '[Type I or Type II]' from the indication heading and clarified wording around diagnosis (added the word 'type' before II) and prior approvals from other entities.

2024-10-09clarified

Policy Summary

PayerCigna

PolicyHereditary Angioedema - Takhzyro Prior Authorization Policy

Policy CodePolicy N/A

Change TypeCriteria clarification and additions

Effective Date

Next Review Date

On This Page

B) Patient is currently receiving Takhzyro prophylaxis

Patient has a diagnosis of HAE type I or type II
[documentation required]
According to the prescriber, the patient has had a favorable clinical response since initiating Takhzyro prophylactic therapy compared with baseline (examples: decrease in HAE acute attack frequency, severity, or duration)
Medication is prescribed by or in consultation with an allergist/immunologist or a physician who specializes in the treatment of HAE or related disorders
If the patient is currently receiving the requested therapy but has not previously received approval of Takhzyro for this indication through the Coverage Review Department, review under criteria for Initial Therapy
Operational note in policy body

Documentation Required

Continuation documentation

For patients currently receiving Takhzyro, document diagnosis of HAE type I or II and a favorable clinical response since initiating therapy. Operational note: if prior approval was obtained outside the Coverage Review Department (i.e., the patient is receiving Takhzyro but has not previously received approval through the Coverage Review Department), the patient must meet the initial therapy criteria.