WAO Guideline for the Management of Hereditary Angioedema

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Abstract

Hereditary Angioedema (HAE) is a rare disease and for this reason proper diagnosis and appropriate therapy are often unknown or not available for physicians and other health care providers. For this reason we convened a group of specialists that focus upon HAE from around the world to develop not only a consensus on diagnosis and management of HAE, but to also provide evidence based grades, strength of evidence and classification for the consensus. Since both consensus and evidence grading were adhered to the document meets criteria as a guideline. The outcome of the guideline is to improve diagnosis and management of patients with HAE throughout the world and to help initiate uniform care and availability of therapies to all with the diagnosis no matter where the residence of the individual with HAE exists.

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Most cited references 134

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

Marco Cicardi, Aleena Banerji, Francisco Bracho … (2010)

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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Nanofiltered C1 inhibitor concentrate for treatment of hereditary angioedema.

Bruce L Zuraw, Paula J Busse, Martha White … (2010)

Hereditary angioedema due to C1 inhibitor deficiency is characterized by recurrent acute attacks of swelling that can be painful and sometimes life-threatening. We conducted two randomized trials to evaluate nanofiltered C1 inhibitor concentrate in the management of hereditary angioedema. The first study compared nanofiltered C1 inhibitor concentrate with placebo for treatment of an acute attack of angioedema. A total of 68 subjects (35 in the C1 inhibitor group and 33 in the placebo group) were given one or two intravenous injections of the study drug (1000 units each). The primary end point was the time to the onset of unequivocal relief. The second study was a crossover trial involving 22 subjects with hereditary angioedema that compared prophylactic twice-weekly injections of nanofiltered C1 inhibitor concentrate (1000 units) with placebo during two 12-week periods. The primary end point was the number of attacks of angioedema per period, with each subject acting as his or her own control. In the first study, the median time to the onset of unequivocal relief from an attack was 2 hours in the subjects treated with C1 inhibitor concentrate but longer than 4 hours in those given placebo (P=0.02). In the second study, the number of attacks per 12-week period was 6.26 with C1 inhibitor concentrate given as prophylaxis, as compared with 12.73 with placebo (P<0.001); the subjects who received the C1 inhibitor concentrate also had significant reductions in both the severity and the duration of attacks, in the need for open-label rescue therapy, and in the total number of days with swelling. In subjects with hereditary angioedema, nanofiltered C1 inhibitor concentrate shortened the duration of acute attacks. When used for prophylaxis, nanofiltered C1 inhibitor concentrate reduced the frequency of acute attacks. (Funded by Lev Pharmaceuticals; ClinicalTrials.gov numbers, NCT00289211, NCT01005888, NCT00438815, and NCT00462709.)

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Author and article information

Contributors

Marcus Maurer

Journal

Journal ID (nlm-ta): World Allergy Organ J

Journal ID (iso-abbrev): World Allergy Organ J

Title: The World Allergy Organization Journal

Publisher: World Allergy Organization

ISSN (Electronic): 1939-4551

Publication date Collection: December 2012

Publication date (Electronic): 15 December 2012

Volume: 5

Issue: 12

Pages: 182-199

Affiliations

[1 ]Department of Medicine, Pediatrics and Graduate Studies, Penn State University, Hershey, PA

[2 ]Center for Pediatric and Juvenile Medicine, J.W. Goethe University, Frankfurt/(Main), Germany

[3 ]Department of Dermatology, Johannes Gutenberg University Mainz, Mainz, Germany

[4 ]Departments of Medicine and Pediatrics, University of Calgary, Calgary, Canada

[5 ]HAEi, Orsay, France

[6 ]3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary

[7 ]Outpatient Group of Recurrent Infections and Laboratory of Immunology, Faculty of Medicine ABC; Department of Dermatology, Faculty of Medicine, University of São Paulo, São Paulo, Brazil

[8 ]Department of Medicine, Campbelltown Hospital, University of Western Sydney, Sydney, New South Wales, Australia

[9 ]Allergy, Asthma and Immunology Associates of Tampa Bay, University of South Florida, Tampa, FL

[10 ]Department of Immunology, Barts Health NHS Trust, London, United Kingdom

[11 ]Allergy and Asthma Specialists, Dallas, TX

[12 ]Department of Dermatology and Allergy, Allergie-Centrum-Charité, Charité-Universitätsmedizin Berlin, Berlin, Germany

[13 ]Department of Medicine, University of Alberta, Edmonton, Canada

[14 ]Division of Allergy, Dept. of Pediatrics, Nippon Medical School, Tokyo, Japan

[15 ]President WAO, Professor of Medicine, Department of Medicine, University of California San Diego and San Diego VA Healthcare

Article

Publisher ID: 1939-4551-5-12-182

DOI: 10.1097/WOX.0b013e318279affa

PMC ID: 3651186

PubMed ID: 23282420

SO-VID: edeeedbe-f78e-4705-b2cb-2cf69118a857

License:

This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

WAO Guideline for the Management of Hereditary Angioedema

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Most cited references 134

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

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

Nanofiltered C1 inhibitor concentrate for treatment of hereditary angioedema.

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