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

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

Autores:

M. Maurer, Department of Dermatology and Allergy, Charite—Universit€atsmedizin Berlin, Berlin, Germany.

M. Magerl, Department of Dermatology and Allergy, Charite—Universit€atsmedizin Berlin, Berlin, Germany.

I. Ansotegui, Department of Allergy and Immunology, Hospital Quironsalud Bizkaia, Bilbao, Spain.

E. Aygoren-Pursun, Center for Children and Adolescents, University Hospital Frankfurt, Frankfurt, Germany.

S. Betschel,Division of Clinical Immunology and Allergy, St. Michael’s Hospital, University of Toronto, Toronto, ON, Canada.

K. Bork, Department of Dermatology, Johannes Gutenberg University Mainz, Mainz, Germany.

T. Bowen, Department of Medicine and Pediatrics, University of Calgary, Calgary, AB, Canada.

H. Balle Boysen, HAEi, Lausanne, Switzerland.

H. Farkas, Hungarian Angioedema Center, 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary.

A. S. Grumach, Clinical Immunology, Faculdade de Medicina ABC, S~ao Paulo, Brazil.

M. Hide, Department of Dermatology, Hiroshima University, Hiroshima, Japan.

C. Katelaris, Department of Medicine, Campbelltown Hospital and Western Sydney University, Sydney, NSW, Australia.

R. Lockey, Department of Internal Medicine, University of South Florida Morsani College of Medicine, Tampa, FL, USA.

H. Longhurst, Department of Clinical Biochemistry and Immunology, Addenbrooke’s Hospital, Cambridge University Hospitals NHS Foundation Trust, UK.

W. R. Lumry, Department of Internal Medicine, Allergy/Immunology Division, Southwestern Medical School, University of Texas, Dallas, TX, USA.

I. Martinez-Saguer, Hemophilia Centre Rhine Main, Moerfelden-Walldorf, Germany.

D. Moldovan, University of Medicine and Pharmacy, T^ırgu Mures, Romania.

A. Nast, Berlin Institute of Health, Department of Dermatology, Venereology und Allergy, Division of Evidence based Medicine (dEBM), Corporate Member of Freie Universit€at Berlin, Humboldt-Universit€at zu Berlin, Charite—Universit€atsmedizin Berlin, Berlin, Germany.

R. Pawankar, Department of Pediatrics, Nippon Medical School, Tokyo, Japan.

P. Potter, Department of Medicine, University of Cape Town, Cape Town, South Africa.

M. Riedl, Department of Medicine, University of California—San Diego, La Jolla, CA, USA.

B. Ritchie, Division of Hematology, University of Alberta, Edmonton, AB, Canada.

L. Rosenwasser, Allergy and Immunology Department, University of Missouri at Kansas City School of Medicine, Kansas City, MO, USA.

M. Sanchez-Borges, Allergy and Clinical Immunology Department, Centro Medico Docente La Trinidad, Caracas, Venezuela.

Y. Zhi, Department of Allergy, Peking Union Medical College Hospital and Chinese Academy of Medical Sciences, Beijing, China.

B. Zuraw, San Diego VA Healthcare, San Diego, CA, USA. Department of Medicine, University of California—San Diego, La Jolla, CA, USA.

T. Craig, Department of Medicine and Pediatrics, Penn State University, Hershey, PA, USA.

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?

Texto completo: https://doi.org/10.1111/all.13384