Triggers of Hereditary Angioedema Attacks
While attacks can occur spontaneously, triggers are known to precipitate HAE episodes in many patients. Common triggers include:
- Emotional stress or anxiety. Stress is thought to be a major trigger.
- Minor injuries, dental procedures or surgery. Local trauma and inflammation may trigger attacks.
- Menstruation: Attacks frequently occur in some women just before or during periods.
- Medications: Estrogen-containing oral contraceptives and ACE inhibitors are known triggers.
- Infections: Viral infections like colds or flu are common precipitants.
Diagnosis of Hereditary Angioedema
The diagnosis of Hereditary Angioedemainvolves laboratory tests to confirm low levels of C1-inhibitor protein or function. Genetic testing can also detect mutations in the C1-inhibitor gene. Making a prompt diagnosis is important since HAE can be fatal if swelling occurs in the larynx. Delay in diagnosis also increases morbidity from unnecessary medical procedures.
Management of HAE Attacks
Treatment focuses on shortening the duration of acute swelling attacks and preventing future attacks. Medications used for acute attacks include:
- C1-esterase inhibitors such as Berinert and Cinryze given intravenously to stop ongoing attacks.
- Icatibant, a bradykinin receptor antagonist given by subcutaneous injection has a rapid onset.
- Ecallantide, a plasma kallikrein inhibitor, is also used for treatment.
Medications like androgens (danazol) and antifibrinolytics (tranexamic acid) given long-term can reduce attack frequency. Patients receive training to self-administer emergency medications at home. Laryngeal attacks require hospitalization, steroids, airway management and monitoring in ICU
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