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      The International/Canadian Hereditary Angioedema Guideline

      review-article
      1 , , 2 , 1 , 3 , 4 , 5 , 6 , 7 , 6 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 19 , 20 , 21 , 22 , 23 , 24 , 7 , 25 , 26 , 27 , 28 , 29 , 30 , 1 , 31 , 32 , 33 , 27 , 34 , 35 , 36 , 37 , 38 , 39
      Allergy, Asthma, and Clinical Immunology : Official Journal of the Canadian Society of Allergy and Clinical Immunology
      BioMed Central
      Hereditary angioedema, Guideline, Recommendations, Pediatrics, Pregnancy, Acute attacks, Short-term prophylaxis, Long-term prophylaxis, Quality of life, Patient registry

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          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.

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          Most cited references137

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

          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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            Clinical practice. Hereditary angioedema.

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              Icatibant, a new bradykinin-receptor antagonist, in hereditary angioedema.

              Hereditary angioedema is characterized by recurrent attacks of angioedema of the skin, larynx, and gastrointestinal tract. Bradykinin is the key mediator of symptoms. Icatibant is a selective bradykinin B2 receptor antagonist. In two double-blind, randomized, multicenter trials, we evaluated the effect of icatibant in patients with hereditary angioedema presenting with cutaneous or abdominal attacks. In the For Angioedema Subcutaneous Treatment (FAST) 1 trial, patients received either icatibant or placebo; in FAST-2, patients received either icatibant or oral tranexamic acid, at a dose of 3 g daily for 2 days. Icatibant was given once, subcutaneously, at a dose of 30 mg. The primary end point was the median time to clinically significant relief of symptoms. A total of 56 and 74 patients underwent randomization in the FAST-1 and FAST-2 trials, respectively. The primary end point was reached in 2.5 hours with icatibant versus 4.6 hours with placebo in the FAST-1 trial (P=0.14) and in 2.0 hours with icatibant versus 12.0 hours with tranexamic acid in the FAST-2 trial (P<0.001). In the FAST-1 study, 3 recipients of icatibant and 13 recipients of placebo needed treatment with rescue medication. The median time to first improvement of symptoms, as assessed by patients and by investigators, was significantly shorter with icatibant in both trials. No icatibant-related serious adverse events were reported. In patients with hereditary angioedema having acute attacks, we found a significant benefit of icatibant as compared with tranexamic acid in one trial and a nonsignificant benefit of icatibant as compared with placebo in the other trial with regard to the primary end point. The early use of rescue medication may have obscured the benefit of icatibant in the placebo trial. (Funded by Jerini; ClinicalTrials.gov numbers, NCT00097695 and NCT00500656.)
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                Author and article information

                Contributors
                betschels@smh.ca
                Journal
                Allergy Asthma Clin Immunol
                Allergy Asthma Clin Immunol
                Allergy, Asthma, and Clinical Immunology : Official Journal of the Canadian Society of Allergy and Clinical Immunology
                BioMed Central (London )
                1710-1484
                1710-1492
                25 November 2019
                25 November 2019
                2019
                : 15
                : 72
                Affiliations
                [1 ]ISNI 0000 0001 2157 2938, GRID grid.17063.33, University of Toronto, ; Toronto, ON Canada
                [2 ]HAE Canada, Notre Dame des Lourdes, MB Canada
                [3 ]ISNI 0000 0004 1936 8331, GRID grid.410356.5, Department of Medicine, , Queen’s University, ; Kingston, ON Canada
                [4 ]ISNI 0000 0004 1936 8390, GRID grid.23856.3a, Department of Medicine, , Laval University, ; Quebec City, QC Canada
                [5 ]ISNI 0000 0001 2288 9830, GRID grid.17091.3e, Division of Allergy and Clinical Immunology, St. Paul’s Hospital, Department of Medicine, , University of British Columbia, ; Vancouver, BC Canada
                [6 ]ISNI 0000 0004 1936 8227, GRID grid.25073.33, Department of Medicine, , McMaster University, ; Hamilton, ON Canada
                [7 ]ISNI 0000 0004 1936 8200, GRID grid.55602.34, Department of Medicine, , Dalhousie University, ; Halifax, NS Canada
                [8 ]ISNI 0000 0001 2182 2255, GRID grid.28046.38, University of Ottawa Medical School, ; Ottawa, ON Canada
                [9 ]ISNI 0000 0004 1936 9721, GRID grid.7839.5, Goethe-Universität Frankfurt am Main, ; Frankfurt am Main, Germany
                [10 ]ISNI 0000 0001 2179 9593, GRID grid.24827.3b, Department of Internal Medicine, , University of Cincinnati, ; Cincinnati, OH USA
                [11 ]ISNI 0000 0001 1941 7111, GRID grid.5802.f, Department of Dermatology, , University Hospital of the Johannes Gutenberg-University of Mainz, ; Mainz, Germany
                [12 ]GRID grid.440081.9, Hospital La Paz Institute for Health Research, ; Madrid, Spain
                [13 ]Department of Internal Medicine, Universita degli Studi di Milano, Ospedale L. Sacco, Milan, Italy
                [14 ]ISNI 0000 0001 2097 4281, GRID grid.29857.31, Departments of Medicine and Pediatrics, , Penn State University, ; Hershey, PA USA
                [15 ]ISNI 0000 0001 0942 9821, GRID grid.11804.3c, 3rd Department of Internal Medicine, Faculty of Medicine, , Semmelweis University, ; Budapest, Hungary
                [16 ]Laboratory of Clinical Immunology, Faculdade de Medicine ABC, Sao Paulo, Brazil
                [17 ]ISNI 0000 0000 9939 5719, GRID grid.1029.a, Campbelltown Hospital, , Western Sydney University, ; New South Wales, Australia
                [18 ]ISNI 0000 0004 0612 2754, GRID grid.439749.4, Addenbrooke’s Hospital, , Cambridge and University College Hospital, ; London, England UK
                [19 ]ISNI 0000 0001 2107 4242, GRID grid.266100.3, University of California, San Diego, ; San Diego, CA USA
                [20 ]ISNI 0000 0000 9335 334X, GRID grid.416064.1, Moncton Hospital, ; Moncton, NB Canada
                [21 ]ISNI 0000 0004 1936 8390, GRID grid.23856.3a, Division 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 ]ISNI 0000 0001 2292 3357, GRID grid.14848.31, Institut de recherches cliniques de Montréal, ; Montreal, QC Canada
                [25 ]Toronto Allergy Group, Toronto, ON Canada
                [26 ]ISNI 0000 0004 1936 7697, GRID grid.22072.35, Southern Alberta Rare Blood and Bleeding Disorders Program, Foothills Medical Centre, , University of Calgary, ; Calgary, AB Canada
                [27 ]ISNI 0000 0000 9130 6822, GRID grid.25055.37, Division of Pediatrics, Faculty of Medicine, , Memorial University, ; St John’s, NF Canada
                [28 ]ISNI 0000 0000 9130 6822, GRID grid.25055.37, Janeway Children’s Health and Rehabilitation Centre, , Memorial University, ; St John’s, NF Canada
                [29 ]ISNI 0000 0004 1936 8884, GRID grid.39381.30, Division of Clinical Immunology and Allergy, Department of Medicine, , Western University, ; London, ON Canada
                [30 ]ISNI 0000 0004 1936 8227, GRID grid.25073.33, Division of Clinical Immunology and Allergy, Department of Medicine, , McMaster University, ; Hamilton, ON Canada
                [31 ]ISNI 0000 0001 2157 2938, GRID grid.17063.33, Department of Medicine, , University of Toronto, ; Oakville, ON Canada
                [32 ]ISNI 0000 0000 9064 4811, GRID grid.63984.30, Department of Immunology, , McGill University Health Centre, ; Montreal, QC Canada
                [33 ]ISNI 0000 0004 1936 8884, GRID grid.39381.30, Department of Medicine, , Western University, ; London, ON Canada
                [34 ]ISNI 0000 0001 2154 235X, GRID grid.25152.31, College of Medicine, , University of Saskatchewan, ; Regina, SK Canada
                [35 ]ISNI 0000 0004 1936 7697, GRID grid.22072.35, Departments of Medicine, Pediatrics and Oncology, , University of Calgary Cumming School of Medicine, ; Calgary, AB Canada
                [36 ]GRID grid.17089.37, Departments of Medicine and Medical Oncology, , University of Alberta, ; Edmonton, AB Canada
                [37 ]L’angio-oedème héréditaire du Québec, Quebec City, QC Canada
                [38 ]ISNI 0000 0001 2288 9830, GRID grid.17091.3e, Department of Medicine, , University of British Columbia, ; Vancouver, BC Canada
                [39 ]ISNI 0000 0004 1936 8331, GRID grid.410356.5, Department of Internal Medicine, , Queen’s University, ; Kingston, ON Canada
                Author information
                http://orcid.org/0000-0002-6631-4438
                Article
                376
                10.1186/s13223-019-0376-8
                6878678
                31788005
                c8d8e462-192b-4b4e-8cf5-fd2da961d8a6
                © The Author(s) 2019

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 20 August 2019
                : 10 October 2019
                Categories
                Review
                Custom metadata
                © The Author(s) 2019

                Immunology
                hereditary angioedema,guideline,recommendations,pediatrics,pregnancy,acute attacks,short-term prophylaxis,long-term prophylaxis,quality of life,patient registry

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