Photo Credit: SaevichMikalai
The following is a summary of “Hereditary Angioedema Attacks in Patients Receiving Long-Term Prophylaxis: A Systematic Review,” published in the November 2024 issue of Allergy and Immunology by Longhurst et al.
Long-term prophylaxis (LTP) reduces the frequency of hereditary angioedema (HAE) attacks. However, attacks in patients on LTP remain poorly understood.
Researchers conducted a retrospective study to evaluate patients with HAE- C1 inhibitor (C1INH) attacks during LTP and the use of on-demand therapy. They also assessed the characteristics of these attacks.
They conducted a systematic search in PubMed for studies on LTP using plasma-derived (pdC1INH), lanadelumab, berotralstat, androgens, or antifibrinolytics in patients with HAE-C1INH and 45 primary studies met the inclusion criteria.
The results showed attack-free rates of 40% for subcutaneous pdC1INH 60 IU/kg twice weekly at 16 weeks and 44% for lanadelumab 300 mg every 2 weeks at 6 months (77% during steady-state). No difference in attack-free rates was observed for berotralstat 150 mg vs placebo at 24 weeks. Subcutaneous and intravenous pdC1INH showed lower average attack severity compared to placebo. The prophylactic effect of lanadelumab and berotralstat was more pronounced in peripheral attacks. Laryngeal attacks accounted for 2%-7% of all attacks, and on-demand therapy was used in 49%-94% of attacks during LTP.
They found that patients receiving LTP experienced attacks in various anatomic locations, including the larynx. On-demand therapy was used for most attacks, highlighting its essential role for all patients with HAE-C1INH.
Source: link.springer.com/article/10.1007/s12016-024-09006-1
