Table 2 Summary of recommendations
Recommendation | Level of Evidence and Strength of Recommendation |
Treatment of Acute Angioedema Attacks | |
1. Effective therapy should be used to treat acute attacks of angioedema to reduce duration and severity of attacks. | High, Strong |
2. pdC1-INH is an effective therapy for the treatment of acute attacks. | High, Strong |
3. Icatibant is an effective therapy for the treatment of acute attacks. | High, Strong |
4. Ecallantide is an effective therapy for the treatment of acute attacks. | High, Strong |
5. rhC1-INH is an effective therapy for the treatment of acute attacks. | High, Strong |
6. Attenuated androgens should not be used to treat acute attacks. | Low, Strong |
7. Tranexamic acid should not be used to treat acute attacks. | Low, Strong |
8. Frozen plasma could be used for treatment of acute attacks if other recommended therapies are not available. | Low, Strong |
9. We recommend early treatment of attacks to reduce morbidity (Level of Evidence: Moderate) and mortality (Level of Evidence: Expert Opinion). | Moderate, Strong/Expert Opinion, Strong |
10. All attacks of angioedema involving the upper airway are medical emergencies and must be treated immediately. (Level of Evidence: Low) In addition, we recommend emergency department assessment. (Level of Evidence: Expert Opinion). | Low/Expert Opinion, Strong |
Acute Treatment of HAE with Normal C1-INH | |
11. There is insufficient evidence to make a recommendation for or against the use of HAE-specific therapies in the treatment of acute attacks in patients with HAE with normal C1-INH. | Very Low / Insufficient Evidence |
Short-Term Prophylaxis | |
12. Short-term prophylaxis should be considered prior to known patient-specific triggers and for any medical, surgical or dental procedures. | Low, Strong |
13. HAE-specific acute treatment should be available during and after any procedure. | Low, Strong |
Long-Term Prophylaxis In HAE 1 & 2 | |
14. Long-term prophylaxis may be appropriate for some patients to reduce frequency, duration and severity of attacks. | High, Strong |
15. Attenuated androgens are effective for long-term prophylaxis in some patients. | Moderate, Strong |
16. Plasma-derived C1-INH is effective for long-term prophylaxis in some patients. | High, Strong |
17. Anti-fibrinolytics are effective for long-term prophylaxis in some patients. | Moderate, Strong |
18. It is not necessary to fail other long-term prophylaxis therapies before use of C1-INH for long-term prophylaxis is considered. | Expert Opinion, Strong |
19. There is insufficient evidence to make a recommendation for or against long-term prophylaxis for patients with HAE with normal C1-INH. | Very Low/Insufficient Evidence |
Self-Administration | |
20. All 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 | |
21. The decision to start or stop long-term prophylaxis depends on multiple factors and should be made by the patient and an HAE specialist. | Expert Opinion, Strong |
Quality of Life | |
22. Health care providers should specifically address factors known to affect quality of life with HAE patients. Management of HAE should aim to improve patients’ quality of life. | Low, Strong |
Comprehensive Care | |
23. Comprehensive care should be available for all patients with HAE. | Low, Strong |