Skip to main content

Table 2 Summary of recommendations

From: The International/Canadian Hereditary Angioedema Guideline

Recommendation

Level 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 function

High, 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 possible

Consensus, 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 attacks

High, Strong

4. Intravenous pdC1-INH is an effective therapy for the acute treatment of attacks

High, Strong

5. Icatibant is an effective therapy for the acute treatment of attacks

High, Strong

6. Ecallantide is an effective therapy for the acute treatment of attacks

High, Strong

7. Intravenous rhC1-INH is an effective therapy for the acute treatment of attacks

High, Strong

8. Attenuated androgens should not be used for the acute treatment of attacks

Low, Strong

9. Tranexamic acid should not be used for the acute treatment of attacks

Low, Strong

10. Frozen plasma could be used for acute treatment of attacks if other recommended therapies are not available

Low, 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 immediately

Low, 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 patients

Consensus, 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 free

Consensus, Strong

15. Intravenous pdC1-INH is an effective therapy for the acute treatment of HAE-1/2 attacks in paediatric patients

Moderate, Strong

16. Icatibant is an effective therapy for the acute treatment of HAE-1/2 attacks in paediatric patients

Consensus, Strong

17. Intravenous rhC1-INH is an effective therapy for the acute treatment of HAE-1/2 attacks in paediatric patients

Consensus, Strong

18. Ecallantide is an effective therapy for the acute treatment of HAE-1/2 attacks in adolescent patients

Consensus, 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 performed

Low, 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-INH

Moderate, Strong

21. Icatibant is an effective therapy for the acute treatment of attacks in patients with HAE nC1-INH

Consensus, Strong

Short-term prophylaxis

22. Short-term prophylaxis should be considered prior to known patient-specific triggers and for any medical, surgical or dental procedures

Low, Strong

23. HAE-specific acute treatment should be available during and after any procedure

Low, Strong

24. Intravenous pdC1-INH should be used for short-term prophylaxis in patients with HAE

Consensus, 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 attacks

High, Strong

26. pdC1-INH is an effective therapy for long-term prophylaxis in patients with HAE-1/2

High, Strong

27. Lanadelumab is an effective therapy for long-term prophylaxis in patients with HAE-1/2

High, Strong

28. Subcutaneous C1-INH or lanadelumab should be used as first-line therapy for long-term prophylaxis in patients with HAE-1/2

Consensus, Strong

29. Attenuated androgens and anti-fibrinolytics should not be used as first-line therapy for long-term prophylaxis in patients with HAE-1/2

Consensus, Strong

30. Attenuated androgens are an effective therapy for long-term prophylaxis in some patients with HAE-1/2

Moderate, Strong

31. All patients should have a management plan including immediate access to effective treatment for attacks, even when on prophylaxis

Consensus, Strong

Long-term prophylaxis in pregnant HAE patients

32. When long-term prophylaxis is indicated in pregnancy, pdC1-INH is the treatment of choice

Consensus, Strong

33. Attenuated androgens should not be used during pregnancy or during the breastfeeding period

Consensus, Strong

Long-term prophylaxis in paediatric HAE patients

34. When long-term prophylaxis is indicated in paediatric patients, pdC1-INH is the treatment of choice

Consensus, Strong

35. Androgens should not be used for long-term prophylaxis in paediatric patients

Moderate, 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 therapies

Low, 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 specialist

Consensus, Strong

Quality of life

38. Healthcare providers should routinely assess quality of life in HAE patients using validated instruments in order to optimize HAE management

Consensus, Strong

Comprehensive care

39. Comprehensive care for all patients with HAE should be provided to optimize treatment and outcomes

Consensus, 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 enrolment

Consensus, Strong