HAE international home therapy consensus document

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Abstract

Hereditary angioedema (C1 inhibitor deficiency, HAE) is associated with intermittent swellings which are disabling and may be fatal. Effective treatments are available and these are most useful when given early in the course of the swelling. The requirement to attend a medical facility for parenteral treatment results in delays. Home therapy offers the possibility of earlier treatment and better symptom control, enabling patients to live more healthy, productive lives. This paper examines the evidence for patient-controlled home treatment of acute attacks ('self or assisted administration') and suggests a framework for patients and physicians interested in participating in home or self-administration programmes. It represents the opinion of the authors who have a wide range of expert experience in the management of HAE.

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Most cited references 38

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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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Clinical practice. Hereditary angioedema.

Bruce Zuraw (2008)

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Hereditary and acquired angioedema: Problems and progress: Proceedings of the third C1 esterase inhibitor deficiency workshop and beyond

Angelo Agostoni, Emel Aygören-Pürsün, Karen E Binkley … (2004)

Hereditary angioedema (HAE), a rare but life-threatening condition, manifests as acute attacks of facial, laryngeal, genital, or peripheral swelling or abdominal pain secondary to intra-abdominal edema. Resulting from mutations affecting C1 esterase inhibitor (C1-INH), inhibitor of the first complement system component, attacks are not histamine-mediated and do not respond to antihistamines or corticosteroids. Low awareness and resemblance to other disorders often delay diagnosis; despite availability of C1-INH replacement in some countries, no approved, safe acute attack therapy exists in the United States. The biennial C1 Esterase Inhibitor Deficiency Workshops resulted from a European initiative for better knowledge and treatment of HAE and related diseases. This supplement contains work presented at the third workshop and expanded content toward a definitive picture of angioedema in the absence of allergy. Most notably, it includes cumulative genetic investigations; multinational laboratory diagnosis recommendations; current pathogenesis hypotheses; suggested prophylaxis and acute attack treatment, including home treatment; future treatment options; and analysis of patient subpopulations, including pediatric patients and patients whose angioedema worsened during pregnancy or hormone administration. Causes and management of acquired angioedema and a new type of angioedema with normal C1-INH are also discussed. Collaborative patient and physician efforts, crucial in rare diseases, are emphasized. This supplement seeks to raise awareness and aid diagnosis of HAE, optimize treatment for all patients, and provide a platform for further research in this rare, partially understood disorder.

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Journal

Journal ID (nlm-ta): Allergy Asthma Clin Immunol

Title: Allergy, Asthma, and Clinical Immunology : Official Journal of the Canadian Society of Allergy and Clinical Immunology

Publisher: BioMed Central

ISSN (Print): 1710-1484

ISSN (Electronic): 1710-1492

Publication date Collection: 2010

Publication date (Electronic): 28 July 2010

Volume: 6

Issue: 1

Page: 22

Affiliations

[1 ]Department of Immunology, Barts and the London NHS Trust, London, UK

[2 ]3rd Department of Internal Medicine, Faculty of Medicine, Semmelweis University, Budapest, Hungary

[3 ]Departments of Medicine and Pediatrics, Penn State University, Hershey, Pennsylvania, USA

[4 ]Johann Wolfgang Goethe University, Frankfurt/Main, Germany

[5 ]Department of Immunology, Plymouth Hospitals NHS Trust, UK

[6 ]Dept of Internal Medicin, Ryhov County Hospital, SE-55185 Jönköping, Sweden

[7 ]Department of Dermatology, University Hospital of the Johannes Gutenberg-University of Mainz, Mainz, Germany

[8 ]Department of Medicine, CHU de Grenoble, Grenoble, France

[9 ]Executive Director, HAE International, Denmark

[10 ]Department of Dermatology and Allergy Centre, Odense University Hospital, Denmark

[11 ]Hospital La Paz Health Research Institute, Madrid, Spain

[12 ]Department of Internal Medicine, Universita degli Studi di Milano, Ospedale L. Sacco, Milan, Italy

[13 ]Department of Immunology, Barts and the London NHS Trust, London, UK

[14 ]Department of Immunology, Southmead Hospital, Bristol, UK

[15 ]Department of Immunology, St James' Hospital, Leeds, UK

[16 ]Department of Immunology, Barts and the London NHS Trust, London, UK

[17 ]HAE association, Germany

[18 ]Johann Wolfgang Goethe University, Frankfurt/Main, Germany

[19 ]Academic Medical Center, University of Amsterdam, Amsterdam, Netherlands

[20 ]US HAEA Executive Vice President; US HAEA Patient Registry, USA

[21 ]Johann Wolfgang Goethe University, Frankfurt/Main, Germany

[22 ]HAE Association, France

[23 ]Tel Hashomer, and Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Israel

[24 ]Departments of Medicine and Paediatrics, University of Calgary, Calgary, Alberta, Canada

[25 ]University of California, San Diego, San Diego, California, USA

Article

Publisher ID: 1710-1492-6-22

DOI: 10.1186/1710-1492-6-22

PMC ID: 2922091

PubMed ID: 20667125

SO-VID: f080e1e9-ace3-4958-aa77-e56707dfb4be

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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.

HAE international home therapy consensus document

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Most cited references 38

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

Clinical practice. Hereditary angioedema.

Hereditary and acquired angioedema: Problems and progress: Proceedings of the third C1 esterase inhibitor deficiency workshop and beyond

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