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Hereditary angioedema (HAE) drug therapy

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

Administrative process

Hereditary Angioedema medications are not covered without prior authorization from Pharmacy Administration. 


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 inhibitor (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.


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 or facial 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 kallikrein inhibitor, FDA-approved for the treatment of attacks of hereditary angioedema (HAE) in patients 16 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.


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.

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


C9445Injection, C-1 esterase inhibitor (recombinant), Ruconest, 10 units
J0597Injection, C-1 esterase inhibitor (human), Berinert, 10 units
J0598Injection, C-1 esterase inhibitor (human), Cinryze, 10 units
J1290Injection, ecallantide, 1 mg
J1744Injection, icatibant, 1 mg
J3590Unclassified biologics (Ruconest)


NDC Codes

63833082502Berinert 500 unit kit
42227008105Cinryze 500 unit solution
47783010101Kalbitor 10 mg/mL solution
54092070202Firazyr 30 mg/3 mL, 1 syringe
54092070203Firazyr 30 mg/3 mL, 3 syringes
68012035001Ruconest 2100 unit solution
68012035002Ruconest 2100 unit solution

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


  1. Cinryze prescribing information. ViroPharma Incorporated. August 2014.
  2. Berinert prescribing information. CSL Behring LLC. February 2014.
  3. Kalbitor prescribing information. Dyax Corp. September 2014.
  4. Firazyr prescribing information. Shire Orphan Therapies, Inc. August 2013.
  5. Ruconest prescribing informaiton. Salix Pharmaceuticals, Inc. July 2014.
  6. 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.
  7. Bowen, T. Hereditary angioedema: beyond international consensus – circa December 2010 – The Canadian Society of Allergy and Clinical Immunology Dr. David McCourtie Lecture. Allergy Asthma Clin Immunol 2011;7:1.
  8. 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.
  9. Longhurst HJ, Farkas H, Craig T, et al., HAE international home therapy consensus document. Allergy Asthma Clin Immunol 2010;6:22.

This information is for most, but not all, CoOportunity Health 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.

CoOportunity Health has contracted with HealthPartners Administrators, Inc. to provide claims processing, medical management and certain other administrative services.