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 |