CurrentCignaPolicy IP0756

Hereditary Angioedema - Ekterly

Defines Cigna coverage and prior authorization requirements for Ekterly for treatment of acute hereditary angioedema (HAE) attacks in patients aged 12 and older, including diagnostic and prescriber requirements and product-specific restrictions.

Policy Summary

PayerCigna

PolicyHereditary Angioedema - Ekterly

Policy CodePolicy IP0756

Change TypeNew policy

Effective Date

Next Review Date

Key ActionSubmit prior authorization for Ekterly; approvals are provided for 1 year when criteria are met and prescriber is an allergist/immunologist or HAE specialist.

SourceLink

POLICY UPDATE CHANGES

New policy established for Ekterly (sebetralstat) with coverage and prior authorization criteria for acute HAE attacks.

≥12Minimum age for coverage

1 yearAuthorization duration

ProphylaxisNon-covered use

Allergist/HAE specialistPrescriber requirement

Icatibant trialPlan-specific variations

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

Coverage Criteria for Ekterly (sebetralstat)

FDA-Approved Indication — HAE Due to C1-INH Deficiency (Treatment of Acute Attacks)

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

Overall: Either Initial therapy (A) OR continuation for patients who have previously treated attacks with Ekterly (B)

A) Initial therapy: Patient is ≥ 12 years of age; AND has HAE type I or II confirmed by low C1-INH functional level (< 50% of normal) AND low serum C4 at baseline; AND medication is prescribed by or in consultation with an allergist/immunologist or physician who specializes in HAE; AND preferred product criteria for the requested product are met.Age >=12; C1-INH functional <50%; low C4

A diagnosis of HAE with normal C1-INH (HAE type III) does NOT satisfy this requirement.

B) Patient who has treated previous HAE attacks with Ekterly: Patient has a diagnosis of HAE type I or II; AND per prescriber has had a favorable clinical response to Ekterly; AND medication is prescribed by or in consultation with an allergist/immunologist or physician who specializes in HAE.

If patient is currently receiving Ekterly but has not previously received approval through the Coverage Review Department, evaluate under Initial therapy criteria (A).

Plan-specific criteria (Employer and Individual/Family Plans)

Additional plan-level criteria options — patient meets one of the following:

Plan options: 1) Patient is ≥ 12 years of age and < 18 years of age; OR 2) Patient has previously treated an acute HAE attack with Ekterly; OR 3) Patient has tried generic icatibant (Employer plans) or generic icatibant or Sajazir (Individual/Family plans).Age 12-17 for pediatric option

A previous trial of any icatibant product counts toward the trial requirement.

Ekterly (sebetralstat) for any use other than the treatment of acute hereditary angioedema (HAE) attacks is considered not medically necessary. This exclusion includes, but may not be limited to, indications outside the FDA-approved acute‑attack setting.

Use of Ekterly for prophylaxis of HAE attacks is not covered. The policy cites that data are not available to support prophylactic use and that Ekterly is not indicated for prophylaxis, therefore prophylactic treatment is considered not medically necessary.

Coding and Diagnostic Thresholds

C1-INH functional level — diagnostic threshold

Diagnostic thresholdC1-INH functional level < 50% of normal (baseline), as defined by laboratory reference values — documentation required

Associated lab requirementLow serum C4 at baseline, as defined by laboratory reference values — documentation required

Context of useRequired to confirm HAE type I or II for eligibility for Ekterly initial therapy

Provider Actions, Prior Authorization, and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required for Ekterly. Approvals are provided for 1 year when criteria are met. Because of the specialized skills required for evaluation and diagnosis of patients treated with Ekterly, the medication must be prescribed by or in consultation with a physician who specializes in the condition being treated (for example, an allergist/immunologist or a physician who specializes in HAE). A patient currently receiving Ekterly who previously met initial therapy criteria only needs to meet continuation therapy criteria; if prior criteria were not met through the Coverage Review Department but the patient has previously been treated with Ekterly, initial therapy criteria must be met.

Approval duration: 1 year
Prescriber specialty requirement: allergist/immunologist or physician specializing in HAE or related disorders

Step Therapy

Plan-Specific Prior Steps May Apply

Employer and Individual/Family plans may have plan-specific prior steps before Ekterly is covered. For Employer plans, coverage requires meeting one of: age 12–17, prior treatment of an acute HAE attack with Ekterly, or trial of generic icatibant (a prior trial of any icatibant product counts). For Individual and Family plans, coverage requires meeting one of: age 12–17, prior treatment of an acute HAE attack with Ekterly, or trial of generic icatibant or Sajazir (a prior trial of any icatibant product counts).

Background on Ekterly

Ekterly (sebetralstat) is a plasma kallikrein inhibitor indicated for the treatment of acute hereditary angioedema (HAE) attacks in eligible patients. Coverage under this policy is limited to the FDA‑approved acute‑attack indication and to patients meeting the specified diagnostic, age, and prescriber requirements. The policy does not support use for prophylaxis or other non‑acute indications.

Definitions and Disease Types

HAE types I/II — definition

DefinitionHereditary angioedema (HAE) types I and II are due to C1-INH deficiency and are characterized by low C4 and low C1-INH antigenic or functional levels.

Diagnostic testing recommendedMeasure serum C4 level, C1-INH antigenic level, and C1-INH functional level to distinguish HAE types I/II from other forms.

Purpose of testingConfirms eligibility for on-demand acute HAE treatments including Ekterly when criteria met.

HAE-nlC1INH — definition and policy stance

DefinitionHAE-nlC1INH (HAE with normal C1 inhibitor) refers to hereditary angioedema with normal C1-INH levels (also known as HAE type III).

Coverage implicationA diagnosis of HAE with normal C1-INH does NOT satisfy the Ekterly coverage criteria for treatment of acute attacks under this policy.

Revision History and Policy Changes

01/15/2026Policy createdLatest

New policy established for Ekterly (sebetralstat) with coverage and prior authorization criteria for treatment of acute hereditary angioedema (HAE) attacks; prior authorization required with approvals provided for the duration noted and prescriber specialty requirements specified.