Hereditary Angioedema
Research on hereditary angioedema (HAE), an autosomal dominant disorder caused by C1 esterase inhibitor deficiency leading to recurrent episodes of severe swelling. Studies evaluate attack resolution time, symptom relief, and prevention of attacks.
Quick Answer
What it is
Research on hereditary angioedema (HAE), an autosomal dominant disorder caused by C1 esterase inhibitor deficiency leading to recurrent episodes of severe swelling. Studies evaluate attack resolution time, symptom relief, and prevention of attacks.
Key findings
- Grade A: Time to Symptom Relief (Icatibant (Firazyr))
- Grade A: Primary Symptom Relief Onset (Icatibant (Firazyr))
- Grade A: Cutaneous Attack Resolution (Icatibant (Firazyr))
Safety
- Black box warning.
ℹ️ Quick Facts
Quick Facts: Hereditary Angioedema
- Supplements Studied:2
2 supps · 9 outcomes
Detailed Outcomes
A
Time to Symptom Relief
FAST-3: 2.0 hours to 50%+ symptom reduction vs 19.8 hours placebo (P<0.001). FAST-2: 2.0 hours vs 12.0 hours tranexamic acid (P<0.001).
large↓Improves
A
Primary Symptom Relief Onset
FAST-3: Median onset of primary symptom relief 1.5 hours vs 18.5 hours placebo (P<0.001).
large↓Improves
A
Cutaneous Attack Resolution
Rapid and stable relief from cutaneous attacks demonstrated in Phase III trials.
large↑Improves
A
Abdominal Attack Resolution
Significant reduction in abdominal attack symptoms with rapid onset of action.
large↑Improves
A
Laryngeal Attack Resolution
Effective for potentially life-threatening laryngeal attacks, though emergency medical care still recommended.
large↑Improves
A
Symptom Improvement at 4 Hours
EDEMA3 & EDEMA4 Phase 3 (n=168): Treatment outcome score at 4h significantly greater with ecallantide vs placebo (53.4 vs 8.1, P=0.003 in EDEMA4). Mean symptom complex severity score change -0.8 vs -0.4 placebo (P=0.01). Led to FDA approval.
large↑Improves
A
Time to Symptom Relief
EDEMA3+4 pooled analysis: Benefit apparent within 2 hours of dosing. Maintained through 24 hours. Need for rescue intervention: 14% ecallantide vs 36% placebo within 24h. Earlier treatment associated with better outcomes.
moderate↑Worsens
A
Anaphylaxis Risk
SAFETY CONCERN: Anaphylaxis in 3.9% of patients (10/255) in clinical trials. Most within first hour after injection. Black box warning. Must be administered by healthcare professional. Have anaphylaxis treatment available. Self-administration not recommended.
small↑Worsens
B
Laryngeal Attack Efficacy
Subset analysis of laryngeal attacks: Significant improvement in treatment outcome scores. Potential life-saving for airway-threatening attacks. Limited patient numbers in trials - use with close monitoring for severe attacks.
moderate↑Improves