CurrentViva HealthPolicy IC-0018

Berinert (C1 Esterase Inhibitor, Human) (Intravenous)

Defines prior authorization, initial and renewal clinical criteria, dosing, quantity limits, billing codes, and covered diagnosis for outpatient intravenous Berinert for treatment of acute hereditary angioedema (HAE) attacks.

Policy Summary

PayerViva Health

PolicyBerinert (C1 Esterase Inhibitor, Human) (Intravenous)

Policy CodePolicy IC-0018

Change TypePeriodic review (no material changes)

Effective Date

Next Review Date

Key ActionObtain prior authorization (initial valid 12 weeks, renewable every 12 weeks) and confirm specialist involvement and repeat blood testing to document HAE subtype.

POLICY UPDATE CHANGES

Last Review Date updated to 08/05/2025 and references updated through June 2025.

12 weeksInitial prior authorization duration

1000Maximum billable units per 28 days

20 IU/kgDose per acute attack

D84.1Covered ICD-10 diagnosis

Coverage Summary

This policy covers outpatient intravenous Berinert (C1 esterase inhibitor, human) for the treatment of acute hereditary angioedema (HAE) attacks under a prior authorization program. Coverage is provided with criteria including specialist prescribing or consultation, diagnostic confirmation of the HAE subtype, dosing and quantity limits for outpatient use, and billing/code requirements. Status = CURRENT; Subject = Berinert (C1 Esterase Inhibitor, Human) intravenous.

Continuation / Renewal Criteria

Renewal Criteria

Coverage may be renewed when ALL of the following are met:

Renewal criteria

Patient continues to meet the universal and relevant indication-specific criteria from initial approval
Significant improvement in severity and duration of attacks has been achieved and sustained
Absence of unacceptable toxicity from the drug (e.g., severe hypersensitivity, serious thromboembolic events)
Authorization quantity will account for cumulative amount on-hand to allow treatment of up to 4 attacks per 4 weeks for duration of the authorization

Combination Therapy & Exclusions

Concurrent use of Berinert with other agents indicated for acute HAE attacks (such as icatibant [Firazyr], ecallantide [Kalbitor], or Ruconest) is generally not approved. Requests for combination or duplicate acute-attack therapy may be reviewed and considered on a case-by-case basis but are not routinely authorized. There are no additional explicit exclusions or unproven uses noted beyond this combination therapy restriction.

Applicable Codes

Primary HCPCS code for drug billingHCPCSCovered

J0597

Injection, c-1 esterase inhibitor (human), berinert, 10 units; 1 billable unit = 10 units

NDCs listedNDC

63833-0825-xx	Berinert 500 IU single-dose carton kit (single-dose vial + 10 mL Sterile Water for Injection)
63833-0835-xx	Berinert 500 IU single-dose vial

Covered diagnosis codesICD-10Covered

D84.1

Defects in the complement system

Provider Actions & Billing Rules

Prior Authorization

Prior authorization required

Initial prior authorization is required. Initial authorization is valid for 12 weeks and may be renewed every 12 weeks. When authorizing, the cumulative amount of HAE medication(s) the patient has on-hand will be considered and the authorization will provide a sufficient quantity to allow treatment of up to 4 acute attacks per 4 weeks for the duration of the authorization.

J0597

Documentation Required

Specialist involvement and diagnostic testing

Prescriber must be a specialist or the prescriber must consult with a specialist in allergy, immunology, hematology, pulmonology, or medical genetics. Repeat blood testing to confirm the HAE subtype must be available and include C1‑INH functional and antigenic levels, C4, and C1q testing as applicable.

Documentation Required

Avoidance of HAE triggers documentation

Document that the patient is avoiding potential HAE triggers prior to approval, including estrogen-containing therapies (oral contraceptives and hormone replacement therapy), ACE inhibitors or angiotensin II receptor blockers (ARBs), DPP‑IV inhibitors (e.g., sitagliptin), and neprilysin inhibitors (e.g., sacubitril).

Billing Rule

HCPCS unit billing

Bill using HCPCS code J0597. For J0597, 1 billable unit = 10 IU of Berinert. Adhere to the maximum of 1000 billable units per 28 days.

J0597

Denial Risk

Combination therapy restriction

Combination use with other acute HAE agents (icatibant, ecallantide, Ruconest) is generally not approved and may be denied unless a case-by-case justification is provided and reviewed.

Clinical Evidence & References

Key evidence cited includes the product labeling: Berinert package insert, CSL Behring; September 2021 (accessed June 2025), and clinical studies and consensus guidance such as the I.M.P.A.C.T.2 study and international WAO/EAACI guidelines (2017 update and 2021 revision).

Background

Berinert is FDA-approved for the treatment of acute hereditary angioedema attacks. The policy requires that prescribing be by, or in consultation with, an appropriate specialist and that HAE subtype be confirmed by repeat blood testing (C1‑INH antigenic and functional levels, C4, and C1q as applicable). Dosing for an acute attack is 20 IU/kg IV. Authorization quantities are limited and determined to allow treatment of up to 4 acute attacks per 4 weeks, with prior authorization durations initially for 12 weeks and renewals every 12 weeks.

Definitions / Subtype classifications

Billable unit (J0597)For J0597, 1 billable unit = 10 units (10 IU) of Berinert

HAE I (C1‑INH deficiency)Low C1‑INH antigenic level, low C4 level, low C1‑INH functional level; plus family history of HAE or exclusion of acquired angioedema

HAE II (C1‑INH dysfunction)Normal to elevated C1‑INH antigenic level, low C4 level, low C1‑INH functional level

HAE III (HAE with normal C1‑INH)Normal to near normal C1‑INH antigenic level, C4, and C1‑INH function; repeat testing during an attack excludes HAE I/II; plus either a known HAE‑causing mutation or family history with documented antihistamine nonresponse