Table 1 Treatment recommendations

Vital distress

- Immediately and as soon as possible:

- Administer icatibant (Firazyr®): 30 mg subcutaneously or

- plasma-derived C1-INH (Bérinert®): 20 UI/kg intravenously

- Switch the call to the local SAMU to send French EMS

- Gain control of upper airway

Severe attacks

- Immediately and as soon as possible:

 Laryngeal

- Administer icatibant (Firazyr®): 30 mg subcutaneously or

 Facial

- plasma-derived C1-INH (Bérinert®): 20 UI/kg intravenously

 Abdominal

- If treatments are unavailable at home, switch the call to the local SAMU to send an ambulance headed towards a hospital with specific treatments available or being able to get them by French EMS

- Gain control of upper airway

Non-severe attacks (members, genitals)

- Tranexamic acid: 1 g/6 h except for patients who are breastfeeding or have thromboembolic pathology

Surveillance in all cases

Monitoring by phone 30 min, 1 h, 4 h, 12 h and 24 h after the beginning of the attack

Advice to call back SOS-HAE call centre in case of secondary worsening