Managing Hereditary Angioedema with Normal C1 Inhibitor
Real-world data in HAE with normal C1 inhibitor show that withdrawal of combined oral contraceptives (COC), often with progestins, reduces attacks in HAE-FXII, with adjunct roles for tranexamic acid and lanadelumab.
Researchers found that hereditary angioedema with normal C1 inhibitor is frequently hormone sensitive, based on real-world evidence.
- In HAE-FXII, withdrawal of COC, often with progestins, is associated with marked reductions in attack frequency.
- Adjunctive options include tranexamic acid and lanadelumab for selected subtypes.
Investigators analyzed 116 symptomatic patients through a national hereditary angioedema network to characterize clinical features and management patterns in HAE with normal C1 inhibitor.
Most participants were female and reported predominant abdominal symptoms, with diagnostic delay improving in more recent generations.
REAL-WORLD evidence indicates that hereditary angioedema with normal C1 inhibitor is frequently hormone sensitive.
Author's summary: HAE with normal C1 inhibitor is often hormone sensitive and managed with COC withdrawal and adjunctive therapies.
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