Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for HAE provides up-to-date consensus recommendations for the management of HAE. 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 HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2). The key clinical questions covered by these recommendations are: 1) How should HAE-1/2 be defined and classified?, 2) How should HAE-1/2 be diagnosed?, 3) Should HAE-1/2 patients receive prophylactic and/or on-demand treatment and what treatment options should be used?, 4) Should HAE-1/2 management be different for special HAE-1/2 patient groups such as pregnant/lactating women or children?, and 5) Should HAE-1/2 management incorporate self-administration of therapies and patient support measures? This article is co-published with permission in Allergy and the World Allergy Organization Journal.

Summary. Background: Even though quality of life (QoL) has become a key component of medical care, there is no instrument available that specifically measures QoL after pulmonary embolism (PE). Recently, the Pulmonary Embolism Quality of Life (PEmb-QoL) Questionnaire has been developed to address this gap. Objective: To evaluate the validity of the PEmb-QoL questionnaire. Methods: We distributed the PEmbQoL questionnaire and the Short Form-36 (SF-36) questionnaire twice among consecutive subjects with a history of objectively confirmed acute PE. Internal consistency reliability, test-retest reliability, convergent validity and criterion validity, and correlations between the PEmb-QoL and clinical patient characteristics were assessed using standard-scale construction techniques. Results: Ninety participants completed the questionnaires twice. Internal consistency was adequate (CronbachÕs a 0.62-0.94), as well as test-retest reliability (intra-class correlation coefficients: 0.78-0.94). Furthermore, correlation between the PEmb-QoL questionnaire and the SF-36 questionnaire supported convergent validity. Age, obesity, cardiopulmonary comorbidity, centrally located PE and a family history of venous thromboembolism were shown to be independent determinants of disease-specific QoL. Conclusion: The PEmbQoL questionnaire is a reliable instrument to specifically assess QoL following PE, which is helpful in the identification of patients with decreased QoL following acute PE.

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