C1 INHIBITOR INJ,SOLN
Clinical Criteria Summary
Indication & Clinical Use
- C1 Inhibitor (CINRYZE, HAEGARDA) for Hereditary Angioedema
- Prophylaxis (long-term prophylaxis for HAE attacks or short-term prophylaxis prior to procedures or intubation)
Exclusion Criteria
- Angioedema or abdominal pain not associated with C1 inhibitor deficiency, including hereditary angioedema with normal C1 inhibitor (HAE nC1-INH)
- Concomitant use with another prophylaxis agent for HAE (e.g., berotralstat, donidalorsen, or lanadelumab)
Inclusion Criteria
- Restricted to VA / VA Community Care allergy/immunology provider, dermatology provider, or locally designated expert
- Diagnosis of Hereditary Angioedema Type 1 or 2 as established by laboratory testing
Laboratory & Diagnostic Requirements
- C4 level below the limit of normal
- AND one of the following: C1 inhibitor (C1-INH) antigenic level below the limit of normal OR C1-INH functional level below the limit of normal