The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

<jats:title>Abstract</jats:title><jats:p>Hereditary Angioedema (<jats:styled-content style="fixed-case">HAE</jats:styled-content>) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for <jats:styled-content style="fixed-case">HAE</jats:styled-content> provides up‐to‐date consensus recommendations for the management of <jats:styled-content style="fixed-case">HAE</jats:styled-content>. In the development of this update and revision of the guideline, an international expert panel reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in June 2016 in Vienna. 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 this 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 <jats:styled-content style="fixed-case">HAE</jats:styled-content> with deficient C1‐inhibitor (type 1) and <jats:styled-content style="fixed-case">HAE</jats:styled-content> with dysfunctional C1‐inhibitor (type 2). The key clinical questions covered by these recommendations are: (1) How should <jats:styled-content style="fixed-case">HAE</jats:styled-content>‐1/2 be defined and classified?, (2) How should <jats:styled-content style="fixed-case">HAE</jats:styled-content>‐1/2 be diagnosed?, (3) Should <jats:styled-content style="fixed-case">HAE</jats:styled-content>‐1/2 patients receive prophylactic and/or on‐demand treatment and what treatment options should be used?, (4) Should <jats:styled-content style="fixed-case">HAE</jats:styled-content>‐1/2 management be different for special <jats:styled-content style="fixed-case">HAE</jats:styled-content>‐1/2 patient groups such as pregnant/lactating women or children?, and (5) Should <jats:styled-content style="fixed-case">HAE</jats:styled-content>‐1/2 management incorporate self‐administration of therapies and patient support measures?</jats:p>