What are the current guidelines for the long-term management and prophylaxis of patients with hereditary angioedema?

Long-term management and prophylaxis of hereditary angioedema (HAE) focus on preventing attacks, reducing their frequency and severity, and improving quality of life through tailored treatment plans. UK guidelines recommend individualised prophylaxis using C1 esterase inhibitor (C1-INH) replacement therapy or kallikrein inhibitors for patients with frequent or severe attacks, with consideration of attenuated androgens or antifibrinolytics as alternatives when first-line options are unsuitable ¹.

Prophylactic treatment should be considered for patients experiencing recurrent attacks that significantly impact daily life or those at risk of laryngeal oedema. Long-term prophylaxis aims to maintain adequate plasma levels of C1-INH to prevent bradykinin-mediated swelling ¹.

The recent expert consensus from China (Xu et al., 2025) aligns with these principles, emphasising early initiation of long-term prophylaxis in patients with frequent or severe HAE attacks. It highlights the use of plasma-derived or recombinant C1-INH concentrates as first-line prophylactic agents, with kallikrein inhibitors as effective alternatives. The consensus also stresses the importance of patient education, self-administration training, and regular monitoring to optimise outcomes (Xu et al., 2025).

Both sources agree on the necessity of individualised treatment plans, considering attack frequency, severity, patient preference, and comorbidities. They also recommend on-demand treatment availability for acute attacks despite prophylaxis ¹ (Xu et al., 2025).