CurrentCignaPolicy N/A

Hereditary Angioedema - Ekterly Prior Authorization Policy

This prior authorization policy governs coverage and clinical criteria for Ekterly (sebetralstat tablets) as on-demand treatment of acute hereditary angioedema (HAE) attacks for Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyHereditary Angioedema - Ekterly Prior Authorization Policy

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateJul 7, 2025

Key ActionObtain prior authorization and provide documentation of age ≥12, diagnostic labs (functional C1‑INH <50% and low serum C4), and prescriber specialty or consultation to support approval for up to 1 year.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

≥12minimum patient age for coverage

1 yearapproval duration

Not coveredprophylaxis coverage

Requiredlab confirmation

Coverage criteria for Ekterly (sebetralstat)

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Initial therapy — required elements: i. Patient is ≥ 12 years of age; AND ii. Patient has HAE type I or type II as confirmed by: a) baseline functional C1-INH < 50% of normal (laboratory reference) AND b) baseline low serum C4 (laboratory reference); AND iii. The medication is prescribed by or in consultation with an allergist/immunologist or a physician who specializes in the treatment of HAE or related disorders.Functional C1-INH < 50% of normal

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

FDA‑Approved Indication — Continuation Therapy (patients already treated with Ekterly)

Patient who has treated previous HAE attacks with Ekterly. Approve if the patient meets ALL of the following:

Continuation therapy — required elements: i. Patient has a diagnosis of HAE type I or II; AND ii. According to the prescriber, the patient has had a favorable clinical response with Ekterly treatment (examples: decrease in duration of attacks, quick onset of symptom relief, complete resolution of symptoms, or decrease in attack frequency or severity); AND iii. The medication is prescribed by or in consultation with an allergist/immunologist or a physician who specializes in the treatment of HAE or related disorders.n/a

If patient is currently receiving therapy but lacks prior approval, review under Initial Therapy criteria.

Provider requirements, documentation, and prior authorization

Prior Authorization

Prior Authorization Required for Ekterly

Prior authorization required for Ekterly. Approvals may be provided for up to 1 year when criteria for initial therapy or continuation therapy are met, including age ≥ 12 years.

Approve for up to 1 year when initial or continuation criteria are met

Note

Prescriber Specialty Requirement

Approval requires the medication to be prescribed by or in consultation with an allergist/immunologist or a physician who specializes in hereditary angioedema (HAE) or related disorders. No step‑therapy sequencing is required.

Prescription must be by or in consultation with an allergist/immunologist or HAE specialist
No step-therapy sequencing required

Documentation Required

Required Documentation

Documentation is required and must include laboratory confirmation of HAE due to C1-INH deficiency: baseline functional C1‑INH < 50% of normal and low serum C4 at baseline, as defined by the laboratory reference ranges. Documentation may include chart notes, laboratory reports, prescription records, or other relevant clinical records.

Baseline functional C1‑INH < 50% of normal (per lab reference range) [documentation required]
Baseline low serum C4 (per lab reference range) [documentation required]
Supporting documentation: chart notes, lab reports, prescription claims/receipts, or other clinical records

Denial Risk

Prophylaxis Not Covered

Requests for Ekterly for HAE prophylaxis are considered not medically necessary and will be denied.

Ekterly is not covered for HAE prophylaxis

Denial Risk

Missing Diagnostic Confirmation

Requests missing required diagnostic laboratory documentation (baseline functional C1‑INH <50% and low serum C4) or lacking the minimum age requirement (≥ 12 years) will not meet criteria and may be denied.

Missing baseline functional C1‑INH and/or baseline serum C4 documentation may result in denial
Patient age must be ≥ 12 years

Definitions and clinical terms

HAE type I/II — definition

DefinitionHereditary angioedema due to C1‑INH deficiency, typically confirmed by low serum C4 and low functional or antigenic C1‑INH levels.

Diagnostic testsMeasurement of serum C4, C1‑INH antigenic level, and C1‑INH functional level

Exclusion noteHAE with normal C1‑INH (HAE type III) does NOT satisfy criteria for Ekterly use in this policy

On‑demand (acute) therapy — definition

DefinitionTreatment administered at onset of an HAE attack to minimize morbidity and prevent mortality; patients should have ready access to on‑demand medication.

Guideline recommendationGuidelines recommend patients have and carry medication for treatment of at least two attacks

Coverage contextEkterly is indicated and covered by this policy as on‑demand (acute) therapy for HAE attacks in patients ≥ 12 years

Background

Ekterly (sebetralstat) is a plasma kallikrein inhibitor indicated for treatment of acute hereditary angioedema (HAE) attacks in patients aged 12 years and older. Clinical guidance emphasizes prompt access to on‑demand therapies at onset of an attack; diagnostic testing for C4 and C1‑INH antigenic/functional levels is used to confirm HAE type I/II.