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

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Abstract

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?

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The EAACI/GA(2) LEN/EDF/WAO Guideline for the definition, classification, diagnosis, and management of urticaria: the 2013 revision and update.

T Zuberbier, W. Aberer, R Asero … (2014)

This guideline is the result of a systematic literature review using the 'Grading of Recommendations Assessment, Development and Evaluation' (GRADE) methodology and a structured consensus conference held on 28 and 29 November 2012, in Berlin. It is a joint initiative of the Dermatology Section of the European Academy of Allergy and Clinical Immunology (EAACI), the EU-funded network of excellence, the Global Allergy and Asthma European Network (GA(2) LEN), the European Dermatology Forum (EDF), and the World Allergy Organization (WAO) with the participation of delegates of 21 national and international societies. Urticaria is a frequent, mast cell-driven disease, presenting with wheals, angioedema, or both. The life-time prevalence for acute urticaria is approximately 20%. Chronic spontaneous urticaria and other chronic forms of urticaria do not only cause a decrease in quality of life, but also affect performance at work and school and, as such, are members of the group of severe allergic diseases. This guideline covers the definition and classification of urticaria, taking into account the recent progress in identifying its causes, eliciting factors and pathomechanisms. In addition, it outlines evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

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Hereditary angioedema: new findings concerning symptoms, affected organs, and course.

Jochen Hardt, G. Meng, Petra Staubach … (2006)

Hereditary angioedema (HAE) due to C1 inhibitor deficiency is clinically characterized by relapsing skin swellings, abdominal pain attacks, and life-threatening upper airway obstruction. Our aim was to examine a temporal and spatial pattern of the edema episodes by evaluating the long-term course of hereditary angioedema in order to establish a specific swelling pattern. Data were generated from 221 patients with C1 inhibitor deficiency by asking them about symptoms they experienced during their edema episodes. Documentation was accomplished through the use of standardized questionnaires. A total of 131110 edema episodes were observed. Clinical symptoms started at a mean age of 11.2 (SD 7.7) years. During the following cumulative 5736 years, only 370 (6.5%) symptom-free years occurred. Skin swellings, including extremity, facial, genital, and trunk swellings, and abdominal attacks occurred in 97.4% of all edema episodes of the disease. The other episodes were laryngeal edema (0.9%); edema of the soft palate (0.6%); tongue swellings (0.3%); headache episodes (0.7%); episodes affecting urinary bladder (0.3%), chest (0.2%), muscles (0.4%), joints (0.1%), kidneys (0.1%), and esophagus (0.05%), and were partly combined with other edema episodes. The per-patient analysis and the per-episode analysis revealed markedly discrepant results. On average, women had a more severe course of the disease than men. Patients with early onset of clinical symptoms were affected more severely than those with late onset. The described swelling pattern is specific for HAE and allows a tentative diagnosis based on clinical symptoms and the course of the disease.

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Consensus development methods: a review of best practice in creating clinical guidelines.

N. Black, M. Murphy, D Lamping … (1999)

Although there is debate about the appropriate place of guidelines in clinical practice, guidelines can be seen as one way of assisting clinicians in decision-making. Given the likely diversity of opinion that any group of people may display when considering a topic, methods are needed for organising subjective judgements. Three principal methods (Delphi, nominal group technique, consensus development conference) exist which share the common objective of synthesising judgements when a state of uncertainty exists. To identify the factors that shape and influence the clinical guidelines that emerge from consensus development methods and to make recommendations about best practice in the use of such methods. Five electronic databases were searched: Medline (1966-1996), PsychLIT (1974-1996), Social Science Citation Index (1990-1996), ABI Inform and Sociofile. From the searches and reference lists of articles a total of 177 empirical and review articles were selected for review. The output from consensus development methods may be affected by: the way the task is set (choice of cues, recognition of contextual cues, the focus of the task, the comprehensiveness of the scenarios); the selection of participants (choice of individuals, degree of homogeneity of the group, their background, their number); the selection and presentation of scientific information (format, extent to which its quality and content is assessed); the way any interaction is structured (number of rating rounds, ensuring equitable participation, physical environment for meetings); and the method of synthesising individual judgements (definition of agreement, rules governing outliers, method of mathematical aggregation). Although a considerable amount of research has been carried out, many aspects have not been investigated sufficiently. For the time being at least, advice on those aspects has, therefore, to be based on the user's own commonsense and the experience of those who have used or participated in these methods. Even in the long term, some aspects will not be amenable to scientific study. Meanwhile, adherence to best practice will enhance the validity, reliability and impact of the clinical guidelines produced.

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Author and article information

Contributors

M. Maurer: (View ORCID Profile)

M. Hide: (View ORCID Profile)

C. Katelaris: (View ORCID Profile)

Journal

Title: Allergy

Abbreviated Title: Allergy

Publisher: Wiley

ISSN: 01054538

Publication date Created: August 2018

Publication date (Print): August 2018

Publication date (Electronic): March 12 2018

Volume: 73

Issue: 8

Pages: 1575-1596

Affiliations

[1 ]Department of Dermatology and Allergy; Charité-Universitätsmedizin Berlin; Berlin Germany

[2 ]Department of Allergy and Immunology; Hospital Quironsalud Bizkaia; Bilbao Spain

[3 ]Center for Children and Adolescents; University Hospital Frankfurt; Frankfurt Germany

[4 ]Division of Clinical Immunology and Allergy; St. Michael's Hospital; University of Toronto; Toronto ON Canada

[5 ]Department of Dermatology; Johannes Gutenberg University Mainz; Mainz Germany

[6 ]Department of Medicine and Pediatrics; University of Calgary; Calgary AB Canada

[7 ]HAEi; Lausanne Switzerland

[8 ]Hungarian Angioedema Center; 3rd Department of Internal Medicine; Semmelweis University; Budapest Hungary

[9 ]Clinical Immunology; Faculdade de Medicina ABC; São Paulo Brazil

[10 ]Department of Dermatology; Hiroshima University; Hiroshima Japan

[11 ]Department of Medicine; Campbelltown Hospital and Western Sydney University; Sydney NSW Australia

[12 ]Department of Internal Medicine; University of South Florida Morsani College of Medicine; Tampa FL USA

[13 ]Department of Clinical Biochemistry and Immunology; Addenbrooke's Hospital; Cambridge University Hospitals NHS Foundation Trust; UK

[14 ]Department of Internal Medicine; Allergy/Immunology Division; Southwestern Medical School; University of Texas; Dallas TX USA

[15 ]Hemophilia Centre Rhine Main; Moerfelden-Walldorf Germany

[16 ]University of Medicine and Pharmacy; Tîrgu Mures Romania

[17 ]Berlin Institute of Health; Department of Dermatology, Venereology und Allergy; Division of Evidence based Medicine (dEBM); Corporate Member of Freie Universität Berlin; Humboldt-Universität zu Berlin; Charité-Universitätsmedizin Berlin; Berlin Germany

[18 ]Department of Pediatrics; Nippon Medical School; Tokyo Japan

[19 ]Department of Medicine; University of Cape Town; Cape Town South Africa

[20 ]Department of Medicine; University of California-San Diego; La Jolla CA USA

[21 ]Division of Hematology; University of Alberta; Edmonton AB Canada

[22 ]Allergy and Immunology Department; University of Missouri at Kansas City School of Medicine; Kansas City MO USA

[23 ]Allergy and Clinical Immunology Department; Centro Medico Docente La Trinidad; Caracas Venezuela

[24 ]Department of Allergy; Peking Union Medical College Hospital and Chinese Academy of Medical Sciences; Beijing China

[25 ]San Diego VA Healthcare; San Diego CA USA

[26 ]Department of Medicine and Pediatrics; Penn State University; Hershey PA USA

Article

DOI: 10.1111/all.13384

PubMed ID: 29318628

SO-VID: 5754bce4-de7e-4cd2-9009-aad35fac2e31

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

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Most cited references 185

The EAACI/GA(2) LEN/EDF/WAO Guideline for the definition, classification, diagnosis, and management of urticaria: the 2013 revision and update.

Hereditary angioedema: new findings concerning symptoms, affected organs, and course.

Consensus development methods: a review of best practice in creating clinical guidelines.

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Cited by 139

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