C1 esterase inhibitor (human) (Cinryze)

JCODE: J0598

Office administration/home health

Indications for Prior Authorization

Prophylaxis for angioedema attacks in adolescent and adult patient with Hereditary Angioedema (HAE)

Patients must meet the following criteria for the indications above:


  • Diagnosis of Hereditary Angioedema, with a history of frequent (once per month) or severe attacks (laryngeal/upper airway involvement or impaired daily living).
    • C4 level less than 14 mg/L (normal C4 level 9-36)
    • C1 INH level less than 20 mg/dl (normal 21-39 mg/dl)
    • C1 INH (functional) level less than 72% of the reference range (normal >67%)


  • One of the following:
    • History of failure, intolerance or contraindication to danazol; OR
    • History of failure, intolerance or contraindication to one alkylated androgen such as: oxandrolone, methyltestosterone, oxymetholone, or fluoxymesterone.
  • Must be prescribed by an immunologist, allergist, or rheumatologist
  • Patient must be nine years of age or older

Coverage is not authorized for:

  • acquired angioedema
  • use in patients on ACE inhibitor therapy
  • use in patients on estrogen therapy: contraceptives or replacement therapy


Cinryze 1,000 units can be administered IV every 3 or 4 days for prophylaxis against angioedema attacks in HAE patients. The administration rate is 1 ml per minute.

For short term prophylaxis prior to major dental work or surgery

Cinryze 500 - 1500 units IV one time up to 24 hours prior to the procedure.

Approval Period

Initial approval will be for 3 months.

Renewal for one year with documentation of decrease in the frequency or severity of attacks.


Cinryze is made from human blood. There is a risk of transmitting infectious disease including virus and the Creutzfeldt-Jakob agent.

Severe hypersensitivity reactions including anaphylaxis may occur.

Administration of Cinryze has been associated with clot formation when used at high doses.

Western Health Advantage Pharmacy and Therapeutics Committee

Approved/Revised: January 2010 Reviewed: December 2013