Author(s): Marcus Maurer1 , Markus Magerl1 , Ignacio Ansotegui2 , Emel Aygören-Pürsün3 , Stephen Betschel4 , Konrad Bork5 , Tom Bowen6 , Henrik Balle Boysen7 , Henriette Farkas8 , Anete S. Grumach9 , Michihiro Hide10 , Constance Katelaris11 , Richard Lockey12 , Hilary Longhurst13 , William R. Lumry14 , Inmaculada Martinez-Saguer15 , Dumitru Moldovan16 , Alexander Nast17 , Ruby Pawankar18 , Paul Potter19 , Marc Riedl20 , Bruce Ritchie21 , Lanny Rosenwasser22 , Mario Sánchez-Borges23 , Yuxiang Zhi24 , Bruce Zuraw25 and Timothy Craig26
Hereditary angioedema (HAE) is a rare disease and a serious health problem, globally and for affected patients and their families. The pathophysiological background is primarily a vascular reaction to an overshooting local production of bradykinin. Evidence-based recommendations are needed to inform and guide clinical decision makers. This is the first revision and update of the global guideline for the diagnosis and management of HAE . It was developed by the World Allergy Organization (WAO) in collaboration with the European Academy of Allergy and Clinical Immunology (EAACI). This revised and updated WAO/EAACI guideline on the diagnosis and management of HAE differs from previous consensus reports and position papers [2-16]. It results from a complete review of the underlying evidence based on systematic and transparent assessments of the quality of this evidence. We used an approach oriented along the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach for the revision and update of the recommendations provided by this guideline . GRADE is recommended by the World Health Organization (WHO) and takes into account that evidence alone is insufficient, and that values and preferences, clinical circumstances as well as clinical expertise inevitably influence decisions.
During the planning of the WAO HAE International Guideline, Dr. Richard Lockey, then President of the WAO, and Dr. Timothy Craig, Chair of the steering committee, requested nominations from WAO Affiliated Allergy and Immunology Associations to appoint members to the steering committee. For this update and revision of the guideline, the guideline's expert panel and author group was complemented by additional HAE experts to account for the global reach of the guideline.
In the development of this update and revision of the guideline, working groups were assigned to review and assess the evidence available to answer the questions, for which the guideline provides recommendations (), and to raise new clinical questions to be addressed by the guideline . Based on the assessment of the evidence the panel members, in a consensus conference at the EAACI annual meeting in June 2016 in Vienna, Austria, developed the following recommendations. Using the approach outlined below, recommendations made are strong or weak. Strong recommendations indicate that most physicians would want and only few would not want the recommended course of action, that adherence to the recommendation in clinical practice could be used as a quality criterion and that the recommendation can be adapted as policy in most situations and countries. Weak recommendations should be interpreted to indicate that most but not all physicians would want...