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Berinert — Blue Cross Blue Shield of Alabama

Hereditary angioedema (HAE) due to C1INH deficiency (Type 1 or Type 2)

Initial criteria

  • Diagnosis confirmed by decreased C1-INH antigenic level, C1-INH functional level, and C4 level per Type 1 or Type 2 definitions
  • For Type 1 HAE: decreased C1-INH antigenic and functional level and C4 level AND family history of HAE OR acquired angioedema ruled out (onset <30 years, normal C1q, no underlying disease such as lymphoma or MGUS)
  • For Type 2 HAE: decreased C4 level and decreased C1-INH functional level (antigenic normal or elevated)