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Table 2 Summary of recommendations

From: The International/Canadian Hereditary Angioedema Guideline

RecommendationLevel of evidence and strength of recommendation
Diagnosis of HAE
1. The diagnosis of HAE-1/2 should be made by measuring plasma levels of C4, C1-INH antigen and, when necessary, C1-INH functionHigh, Strong
2. All individuals with a positive family history should be considered to be at risk of HAE and should be screened as early as possibleConsensus, Strong
Acute treatment of HAE-1 and HAE-2
3. Effective therapy should be used for the acute treatment of attacks of angioedema to reduce duration and severity of attacksHigh, Strong
4. Intravenous pdC1-INH is an effective therapy for the acute treatment of attacksHigh, Strong
5. Icatibant is an effective therapy for the acute treatment of attacksHigh, Strong
6. Ecallantide is an effective therapy for the acute treatment of attacksHigh, Strong
7. Intravenous rhC1-INH is an effective therapy for the acute treatment of attacksHigh, Strong
8. Attenuated androgens should not be used for the acute treatment of attacksLow, Strong
9. Tranexamic acid should not be used for the acute treatment of attacksLow, Strong
10. Frozen plasma could be used for acute treatment of attacks if other recommended therapies are not availableLow, Strong
11. Attacks should be treated early to reduce morbidity (level of evidence: moderate) and mortality (level of evidence: consensus)Moderate, Strong/Consensus, Strong
12. All attacks of angioedema involving the upper airway are medical emergencies and must be treated immediatelyLow, Strong
Acute treatment and short-term prophylaxis of HAE in pregnant patients
13. pdC1-INH is the treatment of choice for angioedema attacks in pregnant HAE-1/2 patientsConsensus, Strong
Acute treatment of HAE in paediatric patients
14. All paediatric patients diagnosed with HAE should have access to acute treatment, including those that are symptom freeConsensus, Strong
15. Intravenous pdC1-INH is an effective therapy for the acute treatment of HAE-1/2 attacks in paediatric patientsModerate, Strong
16. Icatibant is an effective therapy for the acute treatment of HAE-1/2 attacks in paediatric patientsConsensus, Strong
17. Intravenous rhC1-INH is an effective therapy for the acute treatment of HAE-1/2 attacks in paediatric patientsConsensus, Strong
18. Ecallantide is an effective therapy for the acute treatment of HAE-1/2 attacks in adolescent patientsConsensus, Strong
Diagnosis of HAE with normal C1-inhibitor
19. If the diagnosis of HAE nC1-INH is suspected, a referral should be made to a physician who has expertise with this condition. Testing for gene variants known to be associated with the condition should be performedLow, Strong
Acute treatment of HAE with normal C1-inhibitor
20. pdC1-INH is an effective therapy for the acute treatment of attacks in patients with HAE nC1-INHModerate, Strong
21. Icatibant is an effective therapy for the acute treatment of attacks in patients with HAE nC1-INHConsensus, Strong
Short-term prophylaxis
22. Short-term prophylaxis should be considered prior to known patient-specific triggers and for any medical, surgical or dental proceduresLow, Strong
23. HAE-specific acute treatment should be available during and after any procedureLow, Strong
24. Intravenous pdC1-INH should be used for short-term prophylaxis in patients with HAEConsensus, Strong
Long-term prophylaxis in HAE-1 and HAE-2
25. Long-term prophylaxis may be appropriate for some patients to reduce frequency, duration, and severity of attacksHigh, Strong
26. pdC1-INH is an effective therapy for long-term prophylaxis in patients with HAE-1/2High, Strong
27. Lanadelumab is an effective therapy for long-term prophylaxis in patients with HAE-1/2High, Strong
28. Subcutaneous C1-INH or lanadelumab should be used as first-line therapy for long-term prophylaxis in patients with HAE-1/2Consensus, Strong
29. Attenuated androgens and anti-fibrinolytics should not be used as first-line therapy for long-term prophylaxis in patients with HAE-1/2Consensus, Strong
30. Attenuated androgens are an effective therapy for long-term prophylaxis in some patients with HAE-1/2Moderate, Strong
31. All patients should have a management plan including immediate access to effective treatment for attacks, even when on prophylaxisConsensus, Strong
Long-term prophylaxis in pregnant HAE patients
32. When long-term prophylaxis is indicated in pregnancy, pdC1-INH is the treatment of choiceConsensus, Strong
33. Attenuated androgens should not be used during pregnancy or during the breastfeeding periodConsensus, Strong
Long-term prophylaxis in paediatric HAE patients
34. When long-term prophylaxis is indicated in paediatric patients, pdC1-INH is the treatment of choiceConsensus, Strong
35. Androgens should not be used for long-term prophylaxis in paediatric patientsModerate, Strong
Self-administration
36. All HAE patients should be trained on self-administration of HAE-specific therapies if they are suitable candidates. If patients cannot self-administer therapy, provisions should be made to ensure timely access to all appropriate therapiesLow, Strong
Approach to individualized therapy
37. The decision to start or stop long-term prophylaxis depends on multiple factors and should be made by the patient and an HAE specialistConsensus, Strong
Quality of life
38. Healthcare providers should routinely assess quality of life in HAE patients using validated instruments in order to optimize HAE managementConsensus, Strong
Comprehensive care
39. Comprehensive care for all patients with HAE should be provided to optimize treatment and outcomesConsensus, Strong
40. All HAE patients should be informed about HAE patient association(s)Consensus, Strong
Registries
41. Physicians should participate in an HAE registry and offer patients enrolmentConsensus, Strong