Managing Hereditary Angioedema with Normal C1 Inhibitor
Real-world data in HAE with normal C1 inhibitor show that COC withdrawal, often with progestins, reduces attacks in HAE-FXII, with adjunct roles for tranexamic acid and lanadelumab.
REAL-WORLD evidence indicates that hereditary angioedema with normal C1 inhibitor is frequently hormone sensitive.
In HAE-FXII, withdrawal of combined oral contraceptives, often with progestins, is associated with marked reductions in attack frequency, and 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.
- Diagnostic delay improved in more recent generations, suggesting increased clinician awareness and access to subtype testing.
- In HAE-FXII, combined oral contraceptive withdrawal showed high effectiveness.
Hereditary angioedema with normal C1 inhibitor is frequently hormone sensitive.
Author's summary: Hereditary angioedema management improves with COC withdrawal.