The International/Canadian Hereditary Angioedema Guideline.

Betschel S1, Badiou J2, Binkley K1, Borici-Mazi R3, Hébert J4, Kanani A5, Keith P6, Lacuesta G7, Waserman S6, Yang B8, Aygören-Pürsün E9, Bernstein J10, Bork K11, Caballero T12, Cicardi M13, Craig T14, Farkas H15, Grumach A16, Katelaris C17, Longhurst H18, Riedl M19, Zuraw B19, Berger M20, Boursiquot JN21, Boysen H22, Castaldo A23, Chapdelaine H24, Connors L7, Fu L25, Goodyear D26, Haynes A27, Kamra P28, Kim H29,30, Lang-Robertson K1, Leith E31, McCusker C32, Moote B33, O'Keefe A27, Othman I34, Poon MC35, Ritchie B36, St-Pierre C37, Stark D38, Tsai E39.

Author information

1: 1University of Toronto, Toronto, ON Canada.
2: HAE Canada, Notre Dame des Lourdes, MB Canada.
3: 3Department of Medicine, Queen's University, Kingston, ON Canada.
4: 4Department of Medicine, Laval University, Quebec City, QC Canada.
5: 5Division of Allergy and Clinical Immunology, St. Paul's Hospital, Department of Medicine, University of British Columbia, Vancouver, BC Canada.
6: 6Department of Medicine, McMaster University, Hamilton, ON Canada.
7: 7Department of Medicine, Dalhousie University, Halifax, NS Canada.
8: 8University of Ottawa Medical School, Ottawa, ON Canada.
9: 9Goethe-Universität Frankfurt am Main, Frankfurt am Main, Germany.
10: 10Department of Internal Medicine, University of Cincinnati, Cincinnati, OH USA.
11: 11Department of Dermatology, University Hospital of the Johannes Gutenberg-University of Mainz, Mainz, Germany.
12: 12Hospital La Paz Institute for Health Research, Madrid, Spain.
13: Department of Internal Medicine, Universita degli Studi di Milano, Ospedale L. Sacco, Milan, Italy.
14: 14Departments of Medicine and Pediatrics, Penn State University, Hershey, PA USA.
15: 153rd Department of Internal Medicine, Faculty of Medicine, Semmelweis University, Budapest, Hungary.
16: Laboratory of Clinical Immunology, Faculdade de Medicine ABC, Sao Paulo, Brazil.
17: 17Campbelltown Hospital, Western Sydney University, New South Wales, Australia.
18: 18Addenbrooke's Hospital, Cambridge and University College Hospital, London, England UK.
19: 19University of California, San Diego, San Diego, CA USA.
20: 20Moncton Hospital, Moncton, NB Canada.
21: 21Division of Allergy and Clinical Immunology, Centre hospitalier universitaire de Québec, Laval University, Quebec City, QC Canada.
22: HAE International (HAEi), Horsens, Denmark.
23: HAE International (HAEi), Fairfax, VA USA.
24: 24Institut de recherches cliniques de Montréal, Montreal, QC Canada.
25: Toronto Allergy Group, Toronto, ON Canada.
26: 26Southern Alberta Rare Blood and Bleeding Disorders Program, Foothills Medical Centre, University of Calgary, Calgary, AB Canada.
27: 27Division of Pediatrics, Faculty of Medicine, Memorial University, St John's, NF Canada.
28: 28Janeway Children's Health and Rehabilitation Centre, Memorial University, St John's, NF Canada.
29: 29Division of Clinical Immunology and Allergy, Department of Medicine, Western University, London, ON Canada.
30: 30Division of Clinical Immunology and Allergy, Department of Medicine, McMaster University, Hamilton, ON Canada.
31: 31Department of Medicine, University of Toronto, Oakville, ON Canada.
32: 32Department of Immunology, McGill University Health Centre, Montreal, QC Canada.
33: 33Department of Medicine, Western University, London, ON Canada.
34: 34College of Medicine, University of Saskatchewan, Regina, SK Canada.
35: 35Departments of Medicine, Pediatrics and Oncology, University of Calgary Cumming School of Medicine, Calgary, AB Canada.
36: 36Departments of Medicine and Medical Oncology, University of Alberta, Edmonton, AB Canada.
37: L'angio-oedème héréditaire du Québec, Quebec City, QC Canada.
38: 38Department of Medicine, University of British Columbia, Vancouver, BC Canada.
39: 39Department of Internal Medicine, Queen's University, Kingston, ON Canada.

Abstract

This is an update to the 2014 Canadian Hereditary Angioedema Guideline with an expanded scope to include the management of hereditary angioedema (HAE) patients worldwide. It is a collaboration of Canadian and international HAE experts and patient groups led by the Canadian Hereditary Angioedema Network. The objective of this guideline is to provide evidence-based recommendations, using the GRADE system, for the management of patients with HAE. This includes the treatment of attacks, short-term prophylaxis, long-term prophylaxis, and recommendations for self-administration, individualized therapy, quality of life, and comprehensive care. New to the 2019 version of this guideline are sections covering the diagnosis and recommended therapies for acute treatment in HAE patients with normal C1-INH, as well as sections on pregnant and paediatric patients, patient associations and an HAE registry. Hereditary angioedema results in random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway. Attacks are associated with significant functional impairment, decreased health-related quality of life, and mortality in the case of laryngeal attacks. Caring for patients with HAE can be challenging due to the complexity of this disease. The care of patients with HAE in Canada, as in many countries, continues to be neither optimal nor uniform. It lags behind some other countries where there are more organized models for HAE management, and greater availability of additional licensed therapeutic options. It is anticipated that providing this guideline to caregivers, policy makers, patients, and advocates will not only optimize the management of HAE, but also promote the importance of individualized care. The primary target users of this guideline are healthcare providers who are managing patients with HAE. Other healthcare providers who may use this guideline are emergency and intensive care physicians, primary care physicians, gastroenterologists, dentists, otolaryngologists, paediatricians, and gynaecologists who will encounter patients with HAE and need to be aware of this condition. Hospital administrators, insurers and policy makers may also find this guideline helpful.

KEYWORDS:

Acute attacks; Guideline; Hereditary angioedema; Long-term prophylaxis; Patient registry; Pediatrics; Pregnancy; Quality of life; Recommendations; Short-term prophylaxis