2017 revision and update of the international WAO/EAACI guideline for the management of HAE

An international expert panel has reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in 2016 in Vienna, Austria. The final version of this update and revision of the guideline incorporates the contributions of a board of expert reviewers and the endorsing societies.

The goal of the guideline update and revision is to provide clinicians and their patients with guidance that will assist them in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2).

These are the recommendations:

#1: We recommend that all patients suspected to have HAE 1/2 are assessed for blood levels of C1-INH function, C1-INH protein, and C4. If any of the levels are abnormally low, the tests should be repeated to con rm the diagnosis of HAE 1/2. (≥90 agreement)

#2: We recommend that all attacks are considered for on-demand treatment. We recommend that any attack affecting or potentially affecting the upper airway is treated. (100% agreement)

#3: We recommend that attacks are treated as early as possible. (100% agreement)

#4: We recommend that HAE attacks are treated with either C1-INH, ecallantide, or icatibant (18/20). (90% agreement)

#5: We recommend that intubation or surgical airway intervention is considered early in progressive upper airway edema. (100% agreement)

#6: We recommend that all patients have sufficient medication for on-demand treatment of two attacks and carry on-demand medication at all times. (100% agreement)

#7: We recommend short-term prophylaxis before procedures that can induce an attack. (100% agreement)

#8: We recommend prophylaxis be considered for patients who face events in life that are associated with increased disease activity. (≥90 agreement)

#9: We recommend that patients are evaluated
for long-term prophylaxis at every visit. Disease burden and patient preference should be taken into consideration. (100% agreement)

#10: We recommend use of C1-inhibitor for first-line long-term prophylaxis. (50-75% agreement (majority vote)

#11: We suggest to use androgens as second-line long-term prophylaxis. (50-75% agreement (majority vote))

#12: We suggest adaptation of long-term prophylaxis in terms of dosage and/or treatment interval as needed to minimize burden of disease. (100% agreement)

#13: We recommend testing children from HAE- affected families be carried out as soon as possible and all offspring of an affected parent be tested. (100% agreement)

#14: We recommend C1-INH be used for treatment of HAE attacks in children under the age of 12. (>90% agreement)

#15: We recommend C1-INH as the preferred therapy for HAE attacks during pregnancy and lactation. (100% agreement)

#16: We recommend that all patients have an action plan. (100% agreement)

#17: We suggest that HAE-speci c comprehensive, integrated care is available for all patients. (100% agreement)

#18: We recommend that all patients who are provided with on-demand treatment licensed for self- administration should be taught to self-administer. (100% agreement)

#19: We recommend that all patients with HAE should be educated about possible triggers, which may induce HAE attacks. (100% agreement)

#20: We recommend that family members of individuals with HAE should be screened for the condition based on:

  • autosomal dominant inheritance
  • delayed diagnosis leads to morbidity and decreased quality of life without appropriate therapy
  • risk of the first angioedema event being fatal due to airway involvement without appropriate therapy (100% agreement)

Authors of the 2017 revision and update of the international WAO/EAACI guideline for the management of HAE are M. Maurer, M. Magerl, I. Ansotegui, E. Aygören-Pürsün, S. Betschel, K. Bork, T. Bowen, H. Balle Boysen, H. Farkas, A. S. Grumach, M. Hide, C. Katelaris, R. Lockey, H. Longhurst, W. R. Lumry, I. Martinez-Saguer, D. Moldovan, A. Nast, R. Pawankar, P. Potter, M. Riedl, B. Ritchie, L. Rosenwasser, M. Sánchez-Borges, Y. Zhi, B. Zuraw, and T. Craig.

See the full text here: https://onlinelibrary.wiley.com/doi/full/10.1111/all.13384.