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Coverage criteria policies

Hereditary angioedema (HAE) drug therapy - Minnesota Health Care Programs

These services may or may not be covered by your HealthPartners plan. Please see your plan documents for your specific coverage information. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage.

Administrative Process

Hereditary Angioedema medications require prior authorization from Pharmacy Administration.

Coverage

Agents approved to treat and prevent hereditary angioedema (HAE) are generally covered for patients diagnosed with HAE, under the care of a specialist, and as follows:

C1 inhibitors (Cinryze®, Berinert®, Ruconest®) are covered:

  1. For treating acute attacks; or,
  2. For prophylaxis, C1 inhibitor is reserved for patients with frequent and severe attacks, inadequately controlled with (or with medical contraindications to) androgen therapy.

Kalbitor® is generally covered for:

  1. Treatment of acute attacks, when administered in a supervised setting due to the risk of anaphylaxis.

Firazyr® is generally covered for:

  1. Treatment of acute attacks in patients diagnosed with hereditary angioedema. Three doses will be approved per authorization.

Haegarda® is generally covered for:

  1. Prophylaxis, reserved for patients with frequent and severe attacks, inadequately controlled with (or with medical contraindications to) androgen therapy.

Definitions

Hereditary angioedema (HAE) is a rare genetic disorder resulting from an inherited deficiency or dysfunction of the C1 inhibitor. It is characterized by recurrent episodes of angioedema, without urticaria or pruritis, which most often affect the skin or the mucosal tissues of the upper respiratory and gastrointestinal tracts.

The exact mechanism(s) that lead to angioedema are not fully understood. The role of C1 inhibitor in regulating the production of the proinflammatory mediator bradykinin appears to be critical in the pathogenesis of HAE. Kallikrein is a mediator in the production of bradykinin.

Effective therapies may replace the inhibitor (administration of C1-inhibitor concentrate or fresh frozen plasma) or increase its hepatic synthesis (administration of androgens). During acute attacks the administration of kallikrein inhibitors may reduce the production of bradykinin.

Cinryze® is a C1 inhibitor, FDA-approved for routine prophylaxis against angioedema attacks in adolescent and adult patients with Hereditary Angioedema (HAE).

Berinert® is a C1 inhibitor, FDA-approved for the treatment of acute abdominal, facial, or laryngeal attacks of hereditary angioedema (HAE) in adult and adolescent patients.

Ruconest® is a C1 inhibitor, FDA-approved for the treatment of acute attacks in adult and adolescent patients with hereditary angioedema (HAE). Effectiveness was not established in HAE patients with laryngeal attacks.

Kalbitor® is a plasma kallikrein inhibitor, FDA-approved for the treatment of acute attacks of hereditary angioedema (HAE) in patients 12 years and older.

Firazyr® is a bradykinin B2 receptor antagonist indicated for treatment of acute attacks of hereditary angioedema (HAE) in adults 18 years of age and older.

Haegarda® is a plasma-derived concentrate of C1 esterase inhibitor (human), indicated for routine prophylaxis to prevent Hereditary Angioedema (HAE) attacks in adolescent and adult patients.

Codes are listed below for informational purposes only, and do not guarantee member coverage or provider reimbursement. The list may not be all-inclusive.

HCPCS Codes

Codes

Description

J0597

Injection, C-1 esterase inhibitor (human), Berinert, 10 units

J0598

Injection, C-1 esterase inhibitor (human), Cinryze, 10 units

J1290

Injection, ecallantide, 1 mg (Kalbitor)

J1744

Injection, icatibant, 1 mg (Firazyr)

J0596

Injection, c-1 esterase inhibitor (recombinant), Ruconest, 10 units

C9015

Injection, C-1 esterase inhibitor (human), Haegarda, 10 units

J3490

Injection, C-1 esterase inhibitor (human), Haegarda, 10 units

NDC Codes

Codes

Description

63833082502

Berinert 500 unit kit

63833083501

Berinert 500 unit vial

42227008105

Cinryze 500 unit solution

47783010101

Kalbitor 10 mg/mL solution

54092070202

Firazyr 30 mg/3 mL, 1 syringe

54092070203

Firazyr 30 mg/3 mL, 3 syringes

68012035001

Ruconest 2100IU solution

68012035002

Ruconest 2100IU solution

6383382802

Haegarda 2000IU solution

6383382902

Haegarda 3000IU solution

CPT Copyright American Medical Association. All rights reserved.  CPT is a registered trademark of the American Medical Association.

Products

This information is for most, but not all, HealthPartners plans. Please read your plan documents to see if your plan has limits or will not cover some items. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage. These coverage criteria may not apply to Medicare Products if Medicare requires different coverage. For more information regarding Medicare coverage criteria or for a copy of a Medicare coverage policy, contact Member Services at 952-883-7979 or 1-800-233-9645.

References

  1. Cinryze prescribing information. ViroPharma Incorporated. September 2014.
  2. Berinert prescribing information. CSL Behring LLC. September 2016.
  3. Kalbitor prescribing information. Dyax Corp. March 2015.
  4. Firazyr prescribing information. Shire Orphan Therapies, Inc. December 2015.
  5. Ruconest prescribing informaiton. Salix Pharmaceuticals, Inc. February 2015.
  6. Haegarda prescribing information. CSL Behring LLC. October 2017.
  7. Cicardi M, Banerji A, Bracho F, et al., Icatibant, a new bradykinin-receptor antagonist, in hereditary angioedema. N Eng J Med 2010;363:532-41.
  8. Bowen, T. Hereditary angioedema: beyond international consensus The Canadian Society of Allergy and Clinical Immunology Dr. David McCourtie Lecture. Allergy Asthma Clin Immunol 2011;7:1.
  9. Bowen T, Cicardi M, Farkas H, et al., 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema. Allergy Asthma Clin Immunol 2010;6(1):24.

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Policy activity

  • 01/01/2017 - Date of origin
  • 11/06/2017 - Effective date
Review date
  • 11/2017
Revision date
  • 11/06/2017

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