C1 esterase inhibitor (human) (Cinryze)

OFFICE ADMINISTRATION / HOME HEALTH

JCODE: J0598

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:

HAE

  • 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%) AND
    • 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

Dosing:

  • 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:

  • Initial approval will be for 3 months
  • Renewal for one year with documentation of decrease in the frequency or severity of attacks

Warnings:

  • 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

 

Last review date: December 2, 2013