This protocol applies to patients with a confirmed diagnosis of acquired angioedema secondary to C1 esterase inhibitor deficiency (AAE-C1-INH) who are experiencing 2 or more attacks per week. Multidisciplinary team (MDT) and relevant specialist input is required to diagnose and treat the underlying cause of AAE.
When attack frequency reaches two or more episodes per week despite management of the underlying cause, additional pharmacological intervention is indicated. The structured regimen for this situation is detailed in the full protocol.
The approach in this setting centres on an intravenous replacement strategy directed at restoring the deficient inhibitor — the complete regimen, sequencing, and clinical decision pathway are available in the full protocol below.