What triggers HAE attacks?

ACE inhibitors (absolute contraindication in HAE), estrogen-containing contraceptives, trauma, dental procedures, stress, and infections are major triggers; ACEi are contraindicated in all HAE types.

When is laryngeal HAE life-threatening?

Laryngeal attacks cause airway obstruction and can be fatal; all patients should carry acute treatment (icatibant, C1-INH, ecallantide); 30-50% of untreated laryngeal attacks are fatal.

What prophylaxis threshold warrants lanadelumab?

≥2 HAE attacks/month, or attacks causing significant QoL impairment, are typical thresholds for lanadelumab 300 mg every 4-8 weeks (extending to 8 weeks in attack-free patients ≥26 weeks).

How does icatibant work acutely?

Icatibant (Firazyr) is a bradykinin B2 receptor antagonist; 30 mg SC injection; onset within 30 minutes; treats acute attacks including abdominal (most common) and laryngeal; can repeat in 6 hours x 2.

What is HAE type 3 (estrogen-dependent)?

HAE type 3 (FXII gene mutation) presents predominantly in women; attacks often triggered by estrogen (OCP, pregnancy); C4, C1-INH normal; bradykinin-targeted treatments are effective.

Hereditary Angioedema Attack Severity and Prophylaxis Assessment

Calculates HAE-QoL score and attack frequency to guide choice between on-demand and long-term prophylaxis (lanadelumab, C1-INH concentrate), including kallikrein inhibitor dosing.

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Frequently Asked Questions

What triggers HAE attacks?

When is laryngeal HAE life-threatening?

What prophylaxis threshold warrants lanadelumab?

How does icatibant work acutely?

What is HAE type 3 (estrogen-dependent)?