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Table 2 Summary of recommendations

From: Canadian hereditary angioedema guideline

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