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Hereditary Angioedema Attacks in Patients Receiving Long-Term Prophylaxis: A Systematic Review
Hilary J Longhurst1, Mauro Cancian2, Vesna Grivcheva-Panovska3
1Department of Immunology, Auckland City Hospital, Te Toka Tumai and University of Auckland, Auckland, New Zealand. hilarylo@adhb.govt.nz.
Clinical Reviews in Allergy & Immunology
|November 7, 2024
Summary
Long-term prophylaxis (LTP) reduces hereditary angioedema (HAE) attacks but does not eliminate them. Patients on LTP still experience attacks, including laryngeal ones, requiring on-demand therapy.
Area of Science:
- Immunology
- Genetics
- Pharmacology
Background:
- Long-term prophylaxis (LTP) is used to reduce hereditary angioedema (HAE) attack frequency.
- Characterizing breakthrough attacks in patients on LTP is crucial for optimizing management.
Approach:
- A systematic review of PubMed-identified studies evaluated LTP use in HAE-C1INH patients.
- Included studies reported on plasma-derived C1 inhibitor (pdC1INH), lanadelumab, berotralstat, androgens, or antifibrinolytics.
- Forty-five primary studies met the inclusion criteria for analysis.
Key Points:
- Attack-free rates varied among LTP agents; lanadelumab showed higher rates during steady-state treatment.
- Prophylactic effects were more significant for peripheral attacks than abdominal or laryngeal ones.
- Laryngeal attacks occurred in 2-7% of cases, and on-demand therapy was used in 49-94% of breakthrough attacks.
Conclusions:
- HAE-C1INH patients on LTP can experience attacks in all locations, including the larynx.
- On-demand therapy remains essential for managing breakthrough HAE attacks, even with prophylaxis.
- Outcomes of on-demand therapy use during LTP were not reported, highlighting a need for further research.
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