This scenario covers patients with hereditary C1 inhibitor deficiency (HAE type 1/2) whose disease activity is high and whose attacks are no longer adequately controlled by on-demand therapy alone, making a preventive treatment strategy necessary.
The patient has high hereditary angioedema disease activity and inadequate control of HAE attacks despite on-demand therapy. Long-term prophylaxis should be individualized and considered in all HAE-1/2 patients, taking into account disease activity, quality of life, available healthcare resources, and failure to achieve adequate control by appropriate on-demand therapy.
For this situation, first-line long-term prophylaxis is recommended. The protocol specifies a set of established preventive agents, with guidance on selection based on individual patient factors. The complete regimen — including which agents are recommended and the conditions under which each applies — is available in the full protocol.
The clinical target is complete disease control: the patient no longer experiences angioedema attacks.
DOI: 10.1111/all.15214