BEROTRALSTAT CAP,ORAL
Clinical Criteria Summary
Criteria for Exclusion (All Drugs: Berotralstat, C1 Inhibitor, Ecallantide, Icatibant, Lanadelumab-flyo)
- Angioedema or abdominal pain not associated with C1 inhibitor deficiency
Criteria for General Inclusion (All Drugs)
- Restricted to VA / VA Community Care allergy/immunology or dermatology provider; or for use in Emergency Medicine/Urgent Care
- Diagnosis of Hereditary Angioedema due to C1 inhibitor deficiency (HAE-C1INH) as established by laboratory testing
Criteria for Severe Acute HAE-C1INH Attacks
- Applies to: C1 Inhibitor, Ecallantide, Icatibant
- Symptoms may include: respiratory symptoms or laryngeal involvement, severe abdominal attack (severe pain with nausea and vomiting), significant orofacial swelling
Criteria for Long-Term Prophylaxis
- Applies to: Berotralstat, C1 Inhibitor, Lanadelumab-flyo
- Indicated for HAE-C1INH attacks after consideration of either an attenuated androgen or antifibrinolytic
- Prior use of an attenuated androgen or antifibrinolytic should be discussed with the patient using shared decision-making, including efficacy, routes of administration, and side effects
Criteria for On-Demand Therapy
- Applies to: C1 Inhibitor, Icatibant
- Required for HAE-C1INH attacks despite long-term prophylaxis, or in a patient not receiving long-term prophylaxis
- May be considered prior to implementing long-term prophylaxis and in addition to long-term prophylaxis; need should be determined using shared decision-making
Criteria for Short-Term Prophylaxis
- Applies to: C1 Inhibitor
- Indicated for a major procedure or intubation AND intolerance, contraindication to, or inefficacy with previous trial of attenuated androgens (e.g., danazol)
- Off-label use: Intravenous (IV) formulation preferred; data not available for subcutaneous (SC) C1 Inhibitor