What is the treatment for angioedema?

Treatment of Angioedema

For acute angioedema attacks, first-line treatment includes plasma-derived C1 inhibitor (C1-INH), icatibant, or ecallantide, which should be administered as early as possible to minimize attack duration and severity. 1

Treatment Algorithm Based on Angioedema Type

1. Hereditary Angioedema (HAE) and Bradykinin-Mediated Angioedema

Acute Attack Management:

• First-line medications (in order of preference):

  • Plasma-derived C1-INH (20 IU/kg IV)
  • Icatibant (30 mg subcutaneously in the abdominal area)
  • Ecallantide (administered by healthcare professional)

• Administration timing:

  • Treat as early as possible in the attack
  • Self-administration is recommended when trained
  • Early treatment (within 6 hours) significantly shortens time to symptom relief 1

• Dosing considerations:

  • For icatibant: If response is inadequate, additional 30 mg doses may be administered at intervals of at least 6 hours, not exceeding 3 injections in 24 hours 2
  • For C1-INH: Dosing based on weight (typically 20 IU/kg)

• If first-line treatments unavailable:

  • Fresh frozen plasma (FFP) 10-15 ml/kg
  • Note: FFP carries risks of transfusion reactions, pathogen transmission, and potential symptom worsening 1

Airway Management (Critical):

• All patients with oropharyngeal/laryngeal attacks must be observed in a facility capable of intubation/tracheostomy
• Monitor for signs of impending airway closure:
  • Change in voice
  • Loss of ability to swallow
  • Difficulty breathing
• Consider early elective intubation if signs of airway compromise 1
• Immediate tracheostomy backup must be available

2. Histamine-Mediated Angioedema (Allergic)

• Epinephrine (for severe cases or laryngeal involvement)
• H1 antihistamines
• Corticosteroids
• Removal of trigger if identified

Site-Specific Management

Laryngeal/Oropharyngeal Angioedema:

• Treat as medical emergency
• Administer specific treatment immediately (C1-INH/icatibant/ecallantide for HAE; epinephrine for allergic)
• Observe in medical facility with airway management capabilities
• Avoid direct airway visualization which may worsen edema
• Consider early intubation by skilled personnel if progressive symptoms

Abdominal Attacks:

• Specific HAE medications as above
• Supportive care:
  • Pain control (may require narcotics)
  • Antiemetics for nausea/vomiting
  • Aggressive hydration for third-space fluid sequestration
• Caution: Avoid chronic narcotic use; risk of addiction 1

Extremity/Cutaneous Attacks:

• Specific HAE medications for moderate-severe attacks
• Mild attacks may resolve without specific treatment

Important Considerations

• Self-administration: Patients should be trained to self-administer treatment and carry medication at all times 1
• Treatment timing: Treatment within 6 hours of attack onset significantly improves outcomes 1
• Standard angioedema treatments (epinephrine, corticosteroids, antihistamines) are ineffective for HAE and bradykinin-mediated angioedema 1
• Elderly patients may have increased systemic exposure to icatibant, though specific dosing adjustments are not established 2

Pitfalls to Avoid

• Misdiagnosis: Failure to distinguish between histaminergic and bradykinin-mediated angioedema leads to ineffective treatment
• Delayed treatment: Early intervention is critical, especially for laryngeal attacks
• Inadequate airway monitoring: All patients with oropharyngeal/laryngeal involvement require close observation
• Inappropriate surgical interventions: Abdominal attacks may mimic acute abdomen but rarely require surgery
• Overuse of narcotics: Risk of addiction in patients with frequent attacks

For patients with recurrent angioedema attacks, prophylactic treatment should be considered to reduce attack frequency and severity, particularly before known triggers like dental or surgical procedures.