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Table 60: Hereditary Angioedema Agents

A    B    C    D    E    F    G    H    I    J    K    L    M    N    O    P    Q    R    S    T    U    V    W    X    Y    Z

Drug Category: Complement Inhibitors

Medication Class/Individual Agents: Hereditary Angioedema Agents

I. Prior-Authorization Requirements

 Hereditary Angioedema Agents

Clinical Notes
Drug Details

Drug Generic Name

Drug Brand Name

PA
Status

Drug
Notes
berotralstat Orladeyo PA  
c1 esterase inhibitor, human-Berinert Berinert PA  
c1 esterase inhibitor, human-Cinryze Cinryze PA  
c1 esterase inhibitor, human-Haegarda Haegarda PA  
c1 esterase inhibitor, recombinant-Ruconest Ruconest PA  
ecallantide Kalbitor PA   MB
icatibant Firazyr PA  
lanadelumab-flyo Takhzyro PA  

Please note: In the case where the prior authorization (PA) status column indicates PA, both the brand and generic (if available) require PA. Typically, the generic is preferred when available unless the brand-name drug appears on the MassHealth Brand Name Preferred Over Generic Drug List. In general, when requesting the non-preferred version, whether the brand or generic, the prescriber must provide medical records documenting an inadequate response or adverse reaction to the preferred version, in addition to satisfying the criteria for the drug itself.

  • Berotralstat, Cinryze (c1 esterase inhibitor, human), Haegarda (c1 esterase inhibitor, human), and lanadelumab-flyo are approved for the routine prophylaxis against angioedema attacks in adolescent and adult patients with hereditary angioedema (HAE). Cinryze (c1 esterase inhibitor, human) and Haegarda (c1 esterase inhibitor, human) are also approved in children six years of age and older. 
    • Prophylaxis may be administered short-term in anticipation of a procedure or period of stress, or long-term for the reduction of attack rates. Long-term prophylaxis should be considered based upon multiple parameters, including attack frequency, comorbidities, patient preferences, access to emergency care.1
  • Berinert (c1 esterase inhibitor, human), ecallantide, icatibant, and Ruconest (c1 esterase inhibitor, recombinant) are all FDA approved for the acute attacks of HAE.
  • Berinert (c1 esterase inhibitor, human), Cinryze (c1 esterase inhibitor, human), Haegarda (c1 esterase inhibitor, human), icatibant, lanadelumab-flyo, and Ruconest (c1 esterase inhibitor, recombinant) are approved for patient self-administration after training by a health care professional.
  • Ecallantide is not approved for self- administration and should only be administered by a doctor or nurse with medical support to manage serious allergic reactions and HAE.

 

1Farkas, H,  Zuraw B. Hereditary angioedema (due to C1 inhibitor deficiency): General care and long-term prophylaxis. In Saini S (Ed). UpToDate [database on the internet]. Waltham (MA): UpToDate; 2022 [cited 2022 March 4]. Available from: http://www.utdol.com/utd/index.do.

 
Table Footnotes
MB This drug is available through the health care professional who administers the drug or in an outpatient or inpatient hospital setting. MassHealth does not pay for this drug to be dispensed through the retail pharmacy. If listed, PA does not apply through the hospital outpatient and inpatient settings. Please refer to 130 CMR 433.408 for PA requirements for other health care professionals. Notwithstanding the above, this drug may be an exception to the unified pharmacy policy; please refer to respective MassHealth Accountable Care Partnership Plans (ACPPs) and Managed Care Organizations (MCOs) for PA status and criteria, if applicable.
 

II. Therapeutic Uses

FDA-approved, for example:

  • Treatment of acute attacks of hereditary angioedema (HAE) (Berinert, icatibant, Kalbitor, Ruconest)
  • Prophylaxis against angioedema attacks in patients with HAE (Cinryze, Haegarda, Orladeyo, Takhyzro)

Note: The above list may not include all FDA-approved indications.

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III.  Evaluation Criteria for Approval

Please note: In the case where the prior authorization (PA) status column indicates PA, both the brand and generic (if available) require PA. Typically, the generic is preferred when available unless the brand-name drug appears on the MassHealth Brand Name Preferred Over Generic Drug List. In general, when requesting the non-preferred version, whether the brand or generic, the prescriber must provide medical records documenting an inadequate response or adverse reaction to the preferred version, in addition to satisfying the criteria for the drug itself.

  • All PA requests must include clinical diagnosis, drug name, dose, and frequency.
  • Dispensing in a 90-day supply of medication may be mandated or allowable for agents in this therapeutic class (designated by M90 or A90, respectively, in the Drug Notes section above). Applicable quantity limits are described below as units per day, per month, per 28 days, or as clinically appropriate, and may be extrapolated for fills of longer day supply. 
  • A preferred drug may be designated for this therapeutic class. In general, MassHealth requires a trial of the preferred drug or clinical rationale for prescribing a non-preferred drug within a therapeutic class. Additional information about these agents, including PA requirements and preferred products, can be found within the MassHealth Drug List at www.mass.gov/druglist.
  • For recertification requests, approval may require submission of additional documentation including, but not limited to, documentation of: some or all criteria for the original approval; response to therapy; clinical rationale for continuation of use; status of member’s condition; appropriate diagnosis; appropriate age; appropriate dose, frequency, and duration of use for requested medication; complete treatment plan; current laboratory values; and member’s current weight.
  • Additional criteria may apply depending upon diagnosis and/or requested medication (see below). Other factors, including rebate and FDA-approval status, may change independently of scheduled MassHealth updates, which may result in additional restrictions.

  

Berinert, icatibant, Kalbitor, Ruconest

  • Documentation of all the following is required: 
    • appropriate diagnosis; and
    • prescriber is an allergist or immunologist or consult notes from an allergist or immunologist regarding the diagnosis are provided; and
    • appropriate dosing.
  • For recertification, documentation of the use or expiration of a previously approved product is required.

 

Cinryze, Haegarda, Orladeyo, Takhzyro

  • Documentation of all the following is required:
    • appropriate diagnosis; and
    • prescriber is an allergist or immunologist or consult notes from an allergist or immunologist regarding the diagnosis are provided; and
    • one of the following:
      • member has > one HAE event/30 days; or
      • member has a history of recurrent laryngeal attacks; and
    • appropriate dosing.

 

Original Effective Date: 03/2012

Last Revised Date: 05/2024

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Last updated 02/10/25

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