Pharmacy Prior Authorization Criteria

Hereditary Angioedema Agents (HAE) Quantity Limit Override

Indications for Prior Authorization

Berinert (C1 esterase inhibitor [Human])

• For diagnosis of Acute treatment of Hereditary Angioedema (HAE)
  Indicated for the treatment of acute abdominal, facial, or laryngeal HAE attacks in adult and pediatric patients. The safety and efficacy of Berinert for prophylactic therapy have not been established.

Ekterly (sebetralstat)

• For diagnosis of Acute treatment of Hereditary Angioedema (HAE)
  Indicated for the treatment of acute attacks of HAE in adult and pediatric patients aged 12 years and older.

Firazyr (icatibant), Sajazir (icatibant)

• For diagnosis of Acute treatment of Hereditary Angioedema (HAE)
  Indicated for the treatment of acute attacks of HAE in adults 18 years of age and older.

Kalbitor (ecallantide)

• For diagnosis of Acute treatment of Hereditary Angioedema (HAE)
  Indicated for treatment of acute attacks of HAE in patients 12 years of age and older.

Ruconest (C1 esterase inhibitor [Recombinant])

• For diagnosis of Acute treatment of Hereditary Angioedema (HAE)
  Indicated for the treatment of acute attacks in adult and adolescent patients with HAE. Limitation of Use: Effectiveness was not established in HAE patients with laryngeal attacks.

Criteria

Berinert, Ekterly, Brand Firazyr, generic icatibant, Kalbitor, Ruconest, Sajazir

Quantity Limit Override

Length of Approval: 3 Month(s)

• Prescriber attests patient has 3 or more acute HAE attacks per month (document number of attacks per month if available) [A]
AND
• Prescriber attests patient has been evaluated for the use of prophylactic therapy
AND
• One of the following*:
• For Kalbitor, requested quantity does not exceed the ceiling limit of 48 vials per 30 days. Note to provider: Ceiling limit is based on the quantity sufficient for the treatment of 8 acute attacks per month.
OR
• For Brand Firazyr, generic icatibant, or Sajazir, requested quantity does not exceed the ceiling limit of 24 syringes [72 mL] per 30 days. Note to provider: Ceiling limit is based on the quantity sufficient for the treatment of 8 acute attacks per month.
OR
• For Ruconest, requested quantity does not exceed the ceiling limit of 32 vials per 30 days. Note to provider: Ceiling limit is based on the quantity sufficient for the treatment of 8 acute attacks per month.
OR
• For Berinert, requested quantity does not exceed the ceiling limit of 32 vials per 30 days. Note to provider: Ceiling limit is based on the quantity sufficient for the treatment of 8 acute attacks per month.
OR
• For Ekterly, requested quantity does not exceed the ceiling limit of 32 tablets per 30 days. Note to provider: Ceiling limit is based on the quantity sufficient for the treatment of 8 acute attacks per month.

P & T Revisions

2026-04-14, 2025-07-28, 2024-08-01

References

1. Berinert Prescribing Information. CSL Behring, LLC. Kankakee, IL. September 2021.
2. Ruconest Prescribing Information. Pharming Healthcare Inc. Warren, NJ. April 2020.
3. Firazyr Prescribing Information. Takeda Pharmaceuticals America, Inc. Cambridge, MA. June 2025.
4. Kalbitor Prescribing Information. Takeda Pharmaceuticals U.S.A, Inc. Cambridge, MA. June 2025.
5. Sajazir Prescribing Information. Cycle Pharmaceuticals Ltd. Cambridge, UK. February 2024.
6. Ekterly Prescribing Information. KalVista Pharmaceuticals, Ltd. Framingham, MA. July 2025.
7. Watt M, Martinez-Saguer I, Simon A, et al. Burden of disease in adult patients with hereditary angioedema: results from a multinational survey. Orphanet J Rare Dis. 2021; 16(1):94.

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End Notes

1. A multinational survey of 260 patients with HAE, of which less than 60% were on HAE prophylactic therapy, reported an average of 11.5 attacks over 6 months (1.92 attacks per month). [7]

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Revision History

• 2026-04-14: Annual Review 2026 - No criteria changes. Background updates.
• 2025-07-28: Addition of new drug Ekterly to criteria.
• 2024-08-01: New HAE QL Override Program

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