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RuconestCareFirst (Caremark)

Treatment of acute attacks in adult and adolescent patients with hereditary angioedema (HAE)

Initial criteria

  • Medication will not be used in combination with any other medication used for the treatment of acute HAE attacks
  • Member has C1 inhibitor deficiency or dysfunction as confirmed by laboratory testing AND meets one of the following: C1 inhibitor (C1‑INH) antigenic level below the lower limit of normal as defined by the laboratory performing the test OR normal C1‑INH antigenic level and a low C1‑INH functional level (functional C1‑INH less than 50% or C1‑INH functional level below the lower limit of normal as defined by the laboratory performing the test)
  • OR member has normal C1 inhibitor as confirmed by laboratory testing AND either (a) has an F12, angiopoietin‑1, plasminogen, kininogen‑1 (KNG1), heparan sulfate‑glucosamine 3‑O‑sulfotransferase 6 (HS3ST6), or myoferlin (MYOF) pathogenic variant as confirmed by genetic testing OR (b) has documented family history of angioedema and angioedema was refractory to a trial of high‑dose antihistamine therapy (cetirizine 40 mg/day or equivalent) for at least one month
  • Other causes of angioedema (e.g., ACE inhibitor‑induced angioedema, estrogen‑related angioedema, allergic angioedema) have been ruled out
  • Prescriber is a specialist or in consultation with a specialist in management of hereditary angioedema

Reauthorization criteria

  • Member continues to meet all requirements in the coverage criteria section
  • Member has experienced a reduction in severity and/or duration of acute attacks
  • Prophylaxis considered based on attack frequency, attack severity, comorbid conditions, and member’s quality of life

Approval duration

12 months