What is the primary recommendation for the triage of Hereditary Angioedema (HAE)?

Triage of Hereditary Angioedema (HAE)

Early treatment of HAE attacks is the primary recommendation for triage, with immediate self-administration of on-demand therapy at the earliest recognition of symptoms, regardless of attack location. 1, 2

Initial Assessment and Airway Management

• Immediately assess for airway compromise as the first critical step in managing any patient with HAE, particularly with oropharyngeal or laryngeal involvement 2
• Patients with laryngeal, tongue, or throat swelling should self-administer treatment and seek emergency care immediately due to potential life-threatening airway obstruction 1, 3
• Consider elective intubation if the patient exhibits signs of impending airway closure, such as change in voice, loss of ability to swallow, or difficulty breathing 2, 3
• Historical mortality rates for untreated laryngeal attacks are approximately 30%, emphasizing the critical nature of rapid intervention 3

Treatment Timing and Location

• All attacks should be treated as early as possible, ideally within 1 hour of symptom onset, as this significantly reduces attack duration and severity 1
• Data from the Icatibant Outcome Survey showed attack duration was significantly shorter when treatment was initiated <1 hour vs ≥1 hour (6.1 vs 16.8 hours; P<.001) 1
• Post hoc analyses of IMPACT1 demonstrated that treatment with plasma-derived C1INH within 6 hours of attack onset shortened time to symptom relief (HR 0.53) and complete resolution (HR 0.73) compared to later treatment 1, 4
• Self-administration of treatment outside healthcare settings is strongly recommended to enable earlier treatment 1, 3

Attack Severity and Treatment Decision

• Any attack with potential to interfere with activities of daily living (ADLs) or become moderate/severe should be treated early with on-demand therapy 1
• The World Allergy Organization/European Academy of Allergy and Clinical Immunology (WAO/EAACI) guidelines state that attack severity should not be a precondition for treatment 1
• All abdominal, facial, oral, and upper airway attacks should receive on-demand treatment regardless of severity 1, 3
• For peripheral attacks (extremities), treatment decisions should be guided by potential disability rather than using a "wait-and-see" approach 3, 5

Specific Treatment Options

• First-line treatment for acute HAE attacks is plasma-derived C1 inhibitor concentrate (1000-2000 U intravenously) 3, 6
• Icatibant (30 mg subcutaneously) is indicated for treatment of acute attacks in adults 18 years and older 6
• Additional doses of icatibant may be administered at intervals of at least 6 hours if response is inadequate or symptoms recur, with no more than 3 doses in 24 hours 6
• Standard treatments for allergic reactions (antihistamines, corticosteroids, epinephrine) are NOT effective for HAE and should not be used as first-line treatment 2, 3

Patient Self-Management

• Patients should be trained on self-administration of on-demand therapy to facilitate early treatment 1, 3
• Patients should carry enough on-demand treatment to manage at least 2 attacks at all times 1
• Self-administration leads to faster treatment (44% treated in <1 hour) compared to healthcare provider administration (22% treated in <1 hour) 1
• All patients should have a contingency plan for emergency procedures and airway management 3

Common Pitfalls to Avoid

• Delaying treatment of acute attacks, especially those involving the airway 3
• Using standard angioedema treatments (antihistamines, corticosteroids, epinephrine) which are ineffective for HAE 2, 3
• Discharging patients with oropharyngeal or laryngeal involvement without adequate observation 2
• Waiting for attacks to become severe before initiating treatment 1, 3
• Failing to recognize that early treatment (within 6 hours) significantly improves outcomes compared to delayed treatment 1, 4